Retroperitoneal tumors (RPTs) are very rare tumors that arise in the large space of the retroperitoneum. About two-third of these tumors are malignant, of which soft tissue sarcoma (STS) is the most common and comprises almost one-third of malignant RPTs. Twenty to thirty percent of RPTs are benign. The retroperitoneal cavity has a very large potential space for tumor enlargement to a very high diameters without causing specific symptoms, especially during the initial phase of tumor enlargement. On diagnosis, the average tumor weight is 15 - 20 kg and tumor diameter is 20 - 25 cm. The most common retroperitoneal sarcoma type is liposarcoma, which account for 20% of all sarcoma types and 40% of all retroperitoneal sarcomas (RPSs). Other less common STS arise in the retroperitoneum include leiomyosarcoma and undifferentiated pleomorphic type. Giant liposarcoma is usually defined either as tumor diameter of 30 cm or more or tumor weight of 20 kg or higher. This specific type of sarcoma is very uncommon, with few case reports published in the English literature. Herein, we present a case of a healthy 33-year-old male patient, who was admitted due to abdominal distension and increased body weight since few months. An abdominopelvic computed tomography (CT) scan demonstrated a giant retroperitoneal mass of almost 40 cm in diameter in its largest dimension, located in the right retroperitoneal space. Ultrasound (US)-guided fine needle biopsy (FNB) was consistent with well differentiated liposarcoma. Surgical resection of the tumor along with the right colon, right ureter and kidney, third and fourth duodenal parts and part of the right iliopsoas muscle was contemplated. Histopathological report revealed well-differentiated liposarcoma of 50 cm in diameter, with foci of dedifferentiation, presented by pleomorphic sarcoma. Surgical margins were microscopically negative.
{"title":"Retroperitoneal Liposarcoma: The Giant Type.","authors":"Subhi Mansour, Nassim Azzam, Yoram Kluger, Safi Khuri","doi":"10.14740/jmc4014","DOIUrl":"https://doi.org/10.14740/jmc4014","url":null,"abstract":"<p><p>Retroperitoneal tumors (RPTs) are very rare tumors that arise in the large space of the retroperitoneum. About two-third of these tumors are malignant, of which soft tissue sarcoma (STS) is the most common and comprises almost one-third of malignant RPTs. Twenty to thirty percent of RPTs are benign. The retroperitoneal cavity has a very large potential space for tumor enlargement to a very high diameters without causing specific symptoms, especially during the initial phase of tumor enlargement. On diagnosis, the average tumor weight is 15 - 20 kg and tumor diameter is 20 - 25 cm. The most common retroperitoneal sarcoma type is liposarcoma, which account for 20% of all sarcoma types and 40% of all retroperitoneal sarcomas (RPSs). Other less common STS arise in the retroperitoneum include leiomyosarcoma and undifferentiated pleomorphic type. Giant liposarcoma is usually defined either as tumor diameter of 30 cm or more or tumor weight of 20 kg or higher. This specific type of sarcoma is very uncommon, with few case reports published in the English literature. Herein, we present a case of a healthy 33-year-old male patient, who was admitted due to abdominal distension and increased body weight since few months. An abdominopelvic computed tomography (CT) scan demonstrated a giant retroperitoneal mass of almost 40 cm in diameter in its largest dimension, located in the right retroperitoneal space. Ultrasound (US)-guided fine needle biopsy (FNB) was consistent with well differentiated liposarcoma. Surgical resection of the tumor along with the right colon, right ureter and kidney, third and fourth duodenal parts and part of the right iliopsoas muscle was contemplated. Histopathological report revealed well-differentiated liposarcoma of 50 cm in diameter, with foci of dedifferentiation, presented by pleomorphic sarcoma. Surgical margins were microscopically negative.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 10","pages":"517-520"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1a/0d/jmc-13-517.PMC9635766.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699774","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-10-01Epub Date: 2022-10-31DOI: 10.14740/jmc4003
Marco Alejandro Jimenez-Ochoa, Maria Margarita Contreras-Serratos, Martha Leticia Gonzalez-Bautista, Constantino Lopez-Macias, Diego Alberto Lozano-Jaramillo
Acute lymphoblastic leukemia (ALL) is an aggressive hematological neoplasm typically more common in children than adults. More prolonged remissions and a potential cure can be achieved if allogeneic hematopoietic stem cell transplantation (allo-HSCT) is performed. Outcomes after allo-HSCT vary significantly among patients, and multiple factors contribute to these outcomes. Isolated extramedullary relapse (iEMR) after allo-HSCT is rare. We present the case of a 43-year-old man who was diagnosed with Philadelphia chromosome-negative (Ph-neg), B-cell ALL and underwent haploidentical allo-HSCT because of high-risk features at diagnosis. One year later, he was admitted to the hospital with facial and peripheral edema, proteinuria, elevated serum creatinine levels, and hypertension. Renal biopsy was performed immediately. Renal infiltration of TdT+ leukemic cells was detected by immunohistochemistry. Bone marrow aspiration, lumbar puncture, and computed tomography (CT) scans were performed to identify other sites of possible relapse. No other sites were identified, and an extramedullary isolated renal relapse was diagnosed. Intensive re-induction with chemotherapy was not possible because of the coronavirus disease 2019 (COVID-19) infection. Six weeks later, a medullary relapse was noted. Medullary infiltration of B-cell ALL after allo-HSCT has a historically poor prognosis; however, iEMR appears to have a better overall prognosis. The optimal treatment for renal iEMR is still a matter of debate.
{"title":"Isolated Renal Relapse in a Post-Allogenic Transplant Adult Patient With Acute Lymphoblastic Leukemia.","authors":"Marco Alejandro Jimenez-Ochoa, Maria Margarita Contreras-Serratos, Martha Leticia Gonzalez-Bautista, Constantino Lopez-Macias, Diego Alberto Lozano-Jaramillo","doi":"10.14740/jmc4003","DOIUrl":"https://doi.org/10.14740/jmc4003","url":null,"abstract":"<p><p>Acute lymphoblastic leukemia (ALL) is an aggressive hematological neoplasm typically more common in children than adults. More prolonged remissions and a potential cure can be achieved if allogeneic hematopoietic stem cell transplantation (allo-HSCT) is performed. Outcomes after allo-HSCT vary significantly among patients, and multiple factors contribute to these outcomes. Isolated extramedullary relapse (iEMR) after allo-HSCT is rare. We present the case of a 43-year-old man who was diagnosed with Philadelphia chromosome-negative (Ph-neg), B-cell ALL and underwent haploidentical allo-HSCT because of high-risk features at diagnosis. One year later, he was admitted to the hospital with facial and peripheral edema, proteinuria, elevated serum creatinine levels, and hypertension. Renal biopsy was performed immediately. Renal infiltration of TdT+ leukemic cells was detected by immunohistochemistry. Bone marrow aspiration, lumbar puncture, and computed tomography (CT) scans were performed to identify other sites of possible relapse. No other sites were identified, and an extramedullary isolated renal relapse was diagnosed. Intensive re-induction with chemotherapy was not possible because of the coronavirus disease 2019 (COVID-19) infection. Six weeks later, a medullary relapse was noted. Medullary infiltration of B-cell ALL after allo-HSCT has a historically poor prognosis; however, iEMR appears to have a better overall prognosis. The optimal treatment for renal iEMR is still a matter of debate.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 10","pages":"499-503"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/5b/70/jmc-13-499.PMC9635765.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699775","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-10-01Epub Date: 2022-10-31DOI: 10.14740/jmc4012
Safi Khuri, Yoram Kluger
Anomalies of the pancreas organ, especially congenital anomalies, are very uncommon, and can involve the pancreatic ductal as well as the pancreatic extra-ductal systems. While these anomalies usually present as an incidental radiological finding during adulthood, sometimes, they can present as recurrent upper abdominal pain as a presentation of recurrent episodes of acute idiopathic pancreatitis. Thus, these anomalies should be regarded in the differential diagnosis list, as a cause, for recurrent idiopathic pancreatitis, especially in the adult age group of patients. Multiple different variations, mainly in duct course and configuration of the pancreatic ductal system have been reported. In addition, duplication anomalies and cystic dilatation of the pancreatic duct are well known anomalies as well. A combined anomaly of the aforementioned anomalies is even rarer. Herein, we present the case of a male patient, 47 years old, with recurrent admissions to different hospitals due to upper abdominal pain and episodes of severe idiopathic acute pancreatitis. Imaging tests, mainly abdomino-pelvic computed tomography (CT) scan and magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP) showed a dominant duct of Santorini without divisum along with cystic dilation of the proximal portion of the Santorini duct. Being a very rare pancreatic duct anomaly, the patient was treated by a multidisciplinary team (MDT) of physicians, including pancreas surgeons, gastroenterologists and radiologists. A surgical resection in the form of total pancreatectomy with Roux-en-Y gastrointestinal reconstruction was contemplated. Perioperative and postoperative periods were uneventful. The previously mentioned anomaly is unknown in the English literature and is introduced as new anomaly known as "Kluri".
{"title":"Kluri Anomaly: Newly Introduced Pancreatic Duct Anomaly.","authors":"Safi Khuri, Yoram Kluger","doi":"10.14740/jmc4012","DOIUrl":"https://doi.org/10.14740/jmc4012","url":null,"abstract":"<p><p>Anomalies of the pancreas organ, especially congenital anomalies, are very uncommon, and can involve the pancreatic ductal as well as the pancreatic extra-ductal systems. While these anomalies usually present as an incidental radiological finding during adulthood, sometimes, they can present as recurrent upper abdominal pain as a presentation of recurrent episodes of acute idiopathic pancreatitis. Thus, these anomalies should be regarded in the differential diagnosis list, as a cause, for recurrent idiopathic pancreatitis, especially in the adult age group of patients. Multiple different variations, mainly in duct course and configuration of the pancreatic ductal system have been reported. In addition, duplication anomalies and cystic dilatation of the pancreatic duct are well known anomalies as well. A combined anomaly of the aforementioned anomalies is even rarer. Herein, we present the case of a male patient, 47 years old, with recurrent admissions to different hospitals due to upper abdominal pain and episodes of severe idiopathic acute pancreatitis. Imaging tests, mainly abdomino-pelvic computed tomography (CT) scan and magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP) showed a dominant duct of Santorini without divisum along with cystic dilation of the proximal portion of the Santorini duct. Being a very rare pancreatic duct anomaly, the patient was treated by a multidisciplinary team (MDT) of physicians, including pancreas surgeons, gastroenterologists and radiologists. A surgical resection in the form of total pancreatectomy with Roux-en-Y gastrointestinal reconstruction was contemplated. Perioperative and postoperative periods were uneventful. The previously mentioned anomaly is unknown in the English literature and is introduced as new anomaly known as \"Kluri\".</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 10","pages":"509-512"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/80/11/jmc-13-509.PMC9635770.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699694","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-10-01Epub Date: 2022-10-31DOI: 10.14740/jmc3986
Peter C Olson, Michael Cinelli, Hamfreth S Rahming, Marc Assaad, Jonathan Spagnola, James C Lafferty
Coronary artery anomalies (CAAs) are known to be anatomical aberrations in the origin and structure. Due to the diverse anatomical variants, surgeons and angiographers have struggled when faced with patients who have CAA. To frame the complicated issues surrounding CAA, we present a case of a young patient found to have two CAAs, concurrently on coronary computed tomography angiography (CCTA), while in the emergency room being evaluated for chest pain. Patient was medically observed without any invasive procedure since he was deemed to have low cardiovascular risk. Subsequently, literature on prevalence, as well as high risk findings are reviewed. Further studies to evaluate pharmacological, angiographic, and surgical interventions may have additional benefit for both patients and practitioners. Our aim is to help shed the light on difficulties cardiologists are facing during angiography. Additionally, our paper offers some guidance for how to evaluate and follow patients with similar findings into the future.
{"title":"Intra-Atrial Right Coronary Artery and Anomalous Origin of Left Circumflex Artery Found Concurrently.","authors":"Peter C Olson, Michael Cinelli, Hamfreth S Rahming, Marc Assaad, Jonathan Spagnola, James C Lafferty","doi":"10.14740/jmc3986","DOIUrl":"https://doi.org/10.14740/jmc3986","url":null,"abstract":"<p><p>Coronary artery anomalies (CAAs) are known to be anatomical aberrations in the origin and structure. Due to the diverse anatomical variants, surgeons and angiographers have struggled when faced with patients who have CAA. To frame the complicated issues surrounding CAA, we present a case of a young patient found to have two CAAs, concurrently on coronary computed tomography angiography (CCTA), while in the emergency room being evaluated for chest pain. Patient was medically observed without any invasive procedure since he was deemed to have low cardiovascular risk. Subsequently, literature on prevalence, as well as high risk findings are reviewed. Further studies to evaluate pharmacological, angiographic, and surgical interventions may have additional benefit for both patients and practitioners. Our aim is to help shed the light on difficulties cardiologists are facing during angiography. Additionally, our paper offers some guidance for how to evaluate and follow patients with similar findings into the future.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 10","pages":"491-494"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/e5/4c/jmc-13-491.PMC9635771.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-10-01Epub Date: 2022-10-31DOI: 10.14740/jmc3979
Mathias Schmandt, Christian Putensen, Tatjana Stiehl, Julia Wagenpfeil, Jens-Christian Schewe, Stefan Felix Ehrentraut
Despite tremendous advances in treatment, acute respiratory distress syndrome (ARDS) remains a disease with high mortality (42-48%). Veno-venous extracorporeal membrane oxygenation (VV-ECMO) is often used as a last treatment option, which poses complex problems for the treatment team, especially with prolonged ECMO support. We report an interesting case of a 40-year-old female patient who developed influenza pneumonia leading to ARDS and subsequently requiring ECMO. Due to severe clotting complications, a prolonged ECMO run time with numerous filter changes was required. After a total of 56 days of ECMO therapy, the patient was successfully weaned. Fortunately, further in the course of treatment, complete recovery with restitutio ad integrum was achieved. A distinguishing feature of this case report is the description of a complete ECMO system change and the concurrent use of two ECMO systems for the same patient. Additionally, we provide data on the patient's current health-related quality of life as measured using the World Health Organization Disability Assessment Schedule 2.0.
{"title":"Be Patient: Prolonged Extracorporeal Membrane Oxygenation Support Including Full System Switch With Favorable Outcome.","authors":"Mathias Schmandt, Christian Putensen, Tatjana Stiehl, Julia Wagenpfeil, Jens-Christian Schewe, Stefan Felix Ehrentraut","doi":"10.14740/jmc3979","DOIUrl":"https://doi.org/10.14740/jmc3979","url":null,"abstract":"<p><p>Despite tremendous advances in treatment, acute respiratory distress syndrome (ARDS) remains a disease with high mortality (42-48%). Veno-venous extracorporeal membrane oxygenation (VV-ECMO) is often used as a last treatment option, which poses complex problems for the treatment team, especially with prolonged ECMO support. We report an interesting case of a 40-year-old female patient who developed influenza pneumonia leading to ARDS and subsequently requiring ECMO. Due to severe clotting complications, a prolonged ECMO run time with numerous filter changes was required. After a total of 56 days of ECMO therapy, the patient was successfully weaned. Fortunately, further in the course of treatment, complete recovery with <i>restitutio ad integrum</i> was achieved. A distinguishing feature of this case report is the description of a complete ECMO system change and the concurrent use of two ECMO systems for the same patient. Additionally, we provide data on the patient's current health-related quality of life as measured using the World Health Organization Disability Assessment Schedule 2.0.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 10","pages":"483-490"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/50/15/jmc-13-483.PMC9635769.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-10-01Epub Date: 2022-10-31DOI: 10.14740/jmc4006
Aldin Malkoc, Kerry E Fine, Ramisa Anjum, Joseph Vivian Davis
Blunt abdominal trauma is associated with a variety of medical complications. Traumatic abdominal wall hernias (TAWHs) are a rare sequela of blunt trauma. Of the various forms of TAWH, a rare subtype described as a "spontaneous lateral ventral hernia" or flank hernia occurs in less than 1% of all blunt abdominal traumas. We present a case of a 39-year-old male with a past medical history of epilepsy who was involved in a rollover motor vehicle collision. It was reported that the patient had a seizure while driving. On physical exam, the patient had a large left lower flank contusion. Computed tomography revealed a complex TAWH with complete avulsion of the abdominal wall musculature from the iliac crest and near to total disruption of the internal oblique. To address this, we used a biological mesh inlay, reinforced with a synthetic Ventralight™ mesh secured to the iliac crest. In this article, we describe the patient's experience and management of a complex TAWH.
{"title":"The Management of Traumatic Abdominal Wall Flank Hernia Along the Spigelian Aponeurosis Using Component Separation, Synthetic, and Biological Mesh.","authors":"Aldin Malkoc, Kerry E Fine, Ramisa Anjum, Joseph Vivian Davis","doi":"10.14740/jmc4006","DOIUrl":"https://doi.org/10.14740/jmc4006","url":null,"abstract":"<p><p>Blunt abdominal trauma is associated with a variety of medical complications. Traumatic abdominal wall hernias (TAWHs) are a rare sequela of blunt trauma. Of the various forms of TAWH, a rare subtype described as a \"spontaneous lateral ventral hernia\" or flank hernia occurs in less than 1% of all blunt abdominal traumas. We present a case of a 39-year-old male with a past medical history of epilepsy who was involved in a rollover motor vehicle collision. It was reported that the patient had a seizure while driving. On physical exam, the patient had a large left lower flank contusion. Computed tomography revealed a complex TAWH with complete avulsion of the abdominal wall musculature from the iliac crest and near to total disruption of the internal oblique. To address this, we used a biological mesh inlay, reinforced with a synthetic Ventralight™ mesh secured to the iliac crest. In this article, we describe the patient's experience and management of a complex TAWH.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 10","pages":"504-508"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/7f/20/jmc-13-504.PMC9635767.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699772","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-09-01Epub Date: 2022-09-28DOI: 10.14740/jmc3973
Armaan Dhaliwal, Vanessa F Eller, Jeffrey J Pu
Hodgkin lymphoma (HL) is a hematologic malignancy that comprises about 10% of all lymphomas with the most common type being classical HL (cHL). The typical clinical presentation of cHL involves multiple region lymphadenopathy and a chest mass found on imaging. However, not all patients present with the typical symptomology of cHL which poses a diagnostic challenge. Extranodal HL, especially primary bone marrow HL (PBMHL), has been described in immunocompromised patients with human immunodeficiency virus (HIV). In this case report, we present a PBMHL case in an immunocompetent patient with no HIV exposure. We discuss a 51-year-old immunocompetent female who presented with 2 - 3 months of fever, confusion, generalized myalgias, and fatigue. She had no lymphadenopathy on physical exam. On further testing, the patient's blood work demonstrated cytopenia and imaging confirmed no lymphadenopathy. Eventually, a bone marrow evaluation established her diagnosis of PBMHL. The patient expired after receiving one cycle of a modified chemotherapy regimen. This case illustrates that HL can be associated with an atypical clinical presentation which may delay diagnosis and treatment. PBMHL can occur in the normal population who is not immunocompromised nor HIV positive. In this situation, the best diagnostic approach is a thorough medical history, physical exam, and bone marrow aspiration and biopsy. Presence of constitutional symptoms without any lymphadenopathy or chest mass should raise the concern for possible atypical HL such as PBMHL. Accurate and timely identification of PBMHL allows for timely initiation of appropriate therapy. While cHL is responsive to chemotherapy, further research is required to improve the therapy for PBMHL.
{"title":"Immunocompetent Patient With Primary Bone Marrow Hodgkin Lymphoma.","authors":"Armaan Dhaliwal, Vanessa F Eller, Jeffrey J Pu","doi":"10.14740/jmc3973","DOIUrl":"https://doi.org/10.14740/jmc3973","url":null,"abstract":"<p><p>Hodgkin lymphoma (HL) is a hematologic malignancy that comprises about 10% of all lymphomas with the most common type being classical HL (cHL). The typical clinical presentation of cHL involves multiple region lymphadenopathy and a chest mass found on imaging. However, not all patients present with the typical symptomology of cHL which poses a diagnostic challenge. Extranodal HL, especially primary bone marrow HL (PBMHL), has been described in immunocompromised patients with human immunodeficiency virus (HIV). In this case report, we present a PBMHL case in an immunocompetent patient with no HIV exposure. We discuss a 51-year-old immunocompetent female who presented with 2 - 3 months of fever, confusion, generalized myalgias, and fatigue. She had no lymphadenopathy on physical exam. On further testing, the patient's blood work demonstrated cytopenia and imaging confirmed no lymphadenopathy. Eventually, a bone marrow evaluation established her diagnosis of PBMHL. The patient expired after receiving one cycle of a modified chemotherapy regimen. This case illustrates that HL can be associated with an atypical clinical presentation which may delay diagnosis and treatment. PBMHL can occur in the normal population who is not immunocompromised nor HIV positive. In this situation, the best diagnostic approach is a thorough medical history, physical exam, and bone marrow aspiration and biopsy. Presence of constitutional symptoms without any lymphadenopathy or chest mass should raise the concern for possible atypical HL such as PBMHL. Accurate and timely identification of PBMHL allows for timely initiation of appropriate therapy. While cHL is responsive to chemotherapy, further research is required to improve the therapy for PBMHL.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 9","pages":"427-431"},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/0c/4a/jmc-13-427.PMC9534194.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40340578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-09-01Epub Date: 2022-09-28DOI: 10.14740/jmc4000
Rasha Aly, Ratna Acharya, Xu Zeng, Kiran Upadhyay
Polyethylene glycol (PEG) 3350, an active ingredient of over-the-counter MiraLAX, is a commonly used laxative in children and is produced by polymerization of ethylene glycol (EG). Masked EG toxicity secondary to contamination of PEG 3350 could occur. We present a 7-year-old child with developmental delay who presented with altered mental status and acute kidney injury (AKI) following intake of generic PEG 3350 for few days prior to presentation. There was high anion gap metabolic acidosis, hypernatremia, elevated osmolar gap, lactic acidosis, and AKI. Urinalysis showed tubular proteinuria, microscopic hematuria, and calcium oxalate crystals. Prior urinalyses were normal without hematuria or proteinuria. Renal biopsy revealed evidence of mesangial dominant immunoglobulin A (IgA) and complement 3 (C3) deposits along with dense tubular deposition of calcium oxalate crystals. He subsequently developed worsening oliguric AKI and required hemodialysis (HD) for several sessions. The AKI resolved within 2 weeks and further HD was not required. Mental status improved in few days. Follow-up urinalyses showed resolution of microscopic hematuria and crystalluria. We hypothesized that the generic PEG 3350 most likely was contaminated with EG leading to the presentation. A high index of suspicion of contamination of PEG 3350 with EG is required in patients presenting with unexplained high anion gap metabolic acidosis, elevated osmolar gap, lactic acidosis, AKI, calcium oxalate crystalluria, and oxalate crystals on renal biopsy. Further studies are needed to determine whether there is an association between transient glomerular mesangial IgA deposition and crystal nephropathy.
{"title":"Polyethylene Glycol 3350 Crystal Nephropathy in Association With Glomerular Mesangial Immunoglobin A Deposition.","authors":"Rasha Aly, Ratna Acharya, Xu Zeng, Kiran Upadhyay","doi":"10.14740/jmc4000","DOIUrl":"https://doi.org/10.14740/jmc4000","url":null,"abstract":"<p><p>Polyethylene glycol (PEG) 3350, an active ingredient of over-the-counter MiraLAX, is a commonly used laxative in children and is produced by polymerization of ethylene glycol (EG). Masked EG toxicity secondary to contamination of PEG 3350 could occur. We present a 7-year-old child with developmental delay who presented with altered mental status and acute kidney injury (AKI) following intake of generic PEG 3350 for few days prior to presentation. There was high anion gap metabolic acidosis, hypernatremia, elevated osmolar gap, lactic acidosis, and AKI. Urinalysis showed tubular proteinuria, microscopic hematuria, and calcium oxalate crystals. Prior urinalyses were normal without hematuria or proteinuria. Renal biopsy revealed evidence of mesangial dominant immunoglobulin A (IgA) and complement 3 (C3) deposits along with dense tubular deposition of calcium oxalate crystals. He subsequently developed worsening oliguric AKI and required hemodialysis (HD) for several sessions. The AKI resolved within 2 weeks and further HD was not required. Mental status improved in few days. Follow-up urinalyses showed resolution of microscopic hematuria and crystalluria. We hypothesized that the generic PEG 3350 most likely was contaminated with EG leading to the presentation. A high index of suspicion of contamination of PEG 3350 with EG is required in patients presenting with unexplained high anion gap metabolic acidosis, elevated osmolar gap, lactic acidosis, AKI, calcium oxalate crystalluria, and oxalate crystals on renal biopsy. Further studies are needed to determine whether there is an association between transient glomerular mesangial IgA deposition and crystal nephropathy.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 9","pages":"475-481"},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/55/7f/jmc-13-475.PMC9534199.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40339576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-09-01Epub Date: 2022-09-28DOI: 10.14740/jmc3963
Asimenia Halioti, Maria Kitinou, Varvara-Maria Chalioti, Georgios Chaliotis
During the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, coronavirus disease 2019 (COVID-19) has been significantly studied for its relationship with diabetes mellitus in general. Still, the association of SARS-CoV-2 infection with diabetic ketoacidosis (DKA) is more specific and warrants a meticulous investigational approach. In this case report, we present a 23-year-old female who developed DKA as the first manifestation of SARS-CoV-2 infection. During hospitalization, the diagnosis of type 1 diabetes mellitus (T1DM) was made and the patient was treated successfully for the metabolic disorder and for SARS-CoV-2. The potential of SARS-CoV-2 to induce DKA in type 1 diabetics is highlighted. We point out that DKA and COVID-19 may have similarities in clinical presentation when gastrointestinal features predominate. In addition, we describe mechanisms that have been hypothesized to explain the negative impact of SARS-CoV-2 on the endocrine pancreatic function.
{"title":"SARS-CoV-2 Unmasks Type 1 Diabetes Mellitus With an Episode of Diabetic Ketoacidosis.","authors":"Asimenia Halioti, Maria Kitinou, Varvara-Maria Chalioti, Georgios Chaliotis","doi":"10.14740/jmc3963","DOIUrl":"https://doi.org/10.14740/jmc3963","url":null,"abstract":"<p><p>During the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, coronavirus disease 2019 (COVID-19) has been significantly studied for its relationship with diabetes mellitus in general. Still, the association of SARS-CoV-2 infection with diabetic ketoacidosis (DKA) is more specific and warrants a meticulous investigational approach. In this case report, we present a 23-year-old female who developed DKA as the first manifestation of SARS-CoV-2 infection. During hospitalization, the diagnosis of type 1 diabetes mellitus (T1DM) was made and the patient was treated successfully for the metabolic disorder and for SARS-CoV-2. The potential of SARS-CoV-2 to induce DKA in type 1 diabetics is highlighted. We point out that DKA and COVID-19 may have similarities in clinical presentation when gastrointestinal features predominate. In addition, we describe mechanisms that have been hypothesized to explain the negative impact of SARS-CoV-2 on the endocrine pancreatic function.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 9","pages":"432-437"},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f3/40/jmc-13-432.PMC9534198.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40340577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nivolumab is a humanized monoclonal anti-programmed cell death receptor-1 (PD-1) antibody that has been authorized for use in the treatment of advanced malignancies. Cutaneous reactions are the most common immune-related adverse events reported with anti-PD-1 agents, and they range broadly from mild localized reactions to rarely severe or life-threatening systemic dermatoses. The occurrence of Steven-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) with nivolumab use is an exceedingly rare phenomenon that was only documented in a handful of cases in the current literature, but it deserves careful attention as SJS/TEN may be associated with fatal outcomes. We present a case of nivolumab-induced SJS/TEN in a middle-aged female patient with metastatic gastric adenocarcinoma that was successfully treated with immunosuppressive therapy and supportive care. Prompt recognition of SJS/TEN with discontinuation of nivolumab is warranted when SJS/TEN is suspected clinically. Multidisciplinary management in a specialized burn unit is the key to improving outcomes of SJS/TEN.
{"title":"Steven-Johnson Syndrome: A Rare but Serious Adverse Event of Nivolumab Use in a Patient With Metastatic Gastric Adenocarcinoma.","authors":"Eltaib Saad, Pabitra Adhikari, Drashti Antala, Ahmed Abdulrahman, Valiko Begiashvili, Khalid Mohamed, Elrazi Ali, Qishou Zhang","doi":"10.14740/jmc3992","DOIUrl":"https://doi.org/10.14740/jmc3992","url":null,"abstract":"<p><p>Nivolumab is a humanized monoclonal anti-programmed cell death receptor-1 (PD-1) antibody that has been authorized for use in the treatment of advanced malignancies. Cutaneous reactions are the most common immune-related adverse events reported with anti-PD-1 agents, and they range broadly from mild localized reactions to rarely severe or life-threatening systemic dermatoses. The occurrence of Steven-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) with nivolumab use is an exceedingly rare phenomenon that was only documented in a handful of cases in the current literature, but it deserves careful attention as SJS/TEN may be associated with fatal outcomes. We present a case of nivolumab-induced SJS/TEN in a middle-aged female patient with metastatic gastric adenocarcinoma that was successfully treated with immunosuppressive therapy and supportive care. Prompt recognition of SJS/TEN with discontinuation of nivolumab is warranted when SJS/TEN is suspected clinically. Multidisciplinary management in a specialized burn unit is the key to improving outcomes of SJS/TEN.</p>","PeriodicalId":16279,"journal":{"name":"Journal of Medical Cases","volume":"13 9","pages":"449-455"},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b7/40/jmc-13-449.PMC9534193.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40339577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}