Pub Date : 2019-11-08DOI: 10.24966/hbtd-2999/100021
Paroxysmal cold haemoglobinuria is an immune haemolytic syndrome characterized by the presence of autoantibodies reactive against specific red blood cell antigens. At low temperatures, these antibodies and the complement fix to the red blood cells, leading to haemolysis upon warming up. We present the case of a twenty-one months old male child, who presented with a six-day history of malaise, productive cough and fe -ver, already medicated with amoxicillin and clavulanic acid two days before. On physical examination, the child was prostrated, severe ly pale, but with no hemodynamic instability or difficulty breathing. Blood tests revealed the presence of severe anaemia (Hb 5.4 g/dL), leucocytosis (19.90 x 10 9 /L), without immature cells in the blood film; reticulocytosis (89.7 x 10 6 /L) and evidence of hemolysis (haptoglo-bin <8 mg/dL; LDH 1328 UI/L; bilirubin 1 mg/dL and unconjugated bilirubin 0.6 mg/dL), C-reactive-protein 56.7 mg/L. He received a transfusion of red blood cells, initiated treatment with oral clarithro mycin and reinforced the rewarming of the extremities with good re - sults (Hb 9.6 g/dL). Regarding serological investigation, the results revealed a positive IgM for Mycoplasma pneumoniae ; positive Direct Antiglobulin Test with specificity for CD3, and positive Donath Land steiner test, leading to the diagnosis. This case report represents a syndrome that even though rare, is important to bear in mind when facing a case of severe haemolysis. Despite the favorable outcome, additional follow-up must be placed in outpatient setting, with periodic clinical and laboratorial revalua -tions.
阵发性寒性血红蛋白尿是一种免疫性溶血综合征，其特征是存在针对特定红细胞抗原的自身抗体。在低温下，这些抗体和补体固定在红细胞上，在升温时导致溶血。我们报告一个21个月大的男婴，他有6天的不适史，咳嗽和发烧，两天前已经服用了阿莫西林和克拉维酸。体格检查时，患儿俯卧，面色严重苍白，但无血流动力学不稳定或呼吸困难。血液检查显示存在严重贫血(血红蛋白5.4 g/dL)，白细胞增多(19.90 x 10 9 /L)，血膜中未见未成熟细胞;网织红细胞增多症(89.7 × 10.6 /L)和溶血症状(haptoglob -bin < 8mg /dL;LDH 1328ui / l;胆红素1 mg/dL和未结合胆红素0.6 mg/dL)， c -反应蛋白56.7 mg/L。他接受了红细胞输注，开始口服克拉霉素治疗，并加强了四肢的复温，结果良好(Hb 9.6 g/dL)。血清学检查结果显示肺炎支原体IgM阳性;CD3特异性直接抗球蛋白试验阳性，Donath Land steiner试验阳性，导致诊断。本病例报告代表了一种综合征，即使罕见，重要的是要记住，当面对严重溶血的情况下。尽管结果良好，但必须在门诊进行额外的随访，定期进行临床和实验室评估。
{"title":"Beware Of Temperature Changes: A Case Report Of Paroxysmal Cold Haemoglobinuria","authors":"","doi":"10.24966/hbtd-2999/100021","DOIUrl":"https://doi.org/10.24966/hbtd-2999/100021","url":null,"abstract":"Paroxysmal cold haemoglobinuria is an immune haemolytic syndrome characterized by the presence of autoantibodies reactive against specific red blood cell antigens. At low temperatures, these antibodies and the complement fix to the red blood cells, leading to haemolysis upon warming up. We present the case of a twenty-one months old male child, who presented with a six-day history of malaise, productive cough and fe -ver, already medicated with amoxicillin and clavulanic acid two days before. On physical examination, the child was prostrated, severe ly pale, but with no hemodynamic instability or difficulty breathing. Blood tests revealed the presence of severe anaemia (Hb 5.4 g/dL), leucocytosis (19.90 x 10 9 /L), without immature cells in the blood film; reticulocytosis (89.7 x 10 6 /L) and evidence of hemolysis (haptoglo-bin <8 mg/dL; LDH 1328 UI/L; bilirubin 1 mg/dL and unconjugated bilirubin 0.6 mg/dL), C-reactive-protein 56.7 mg/L. He received a transfusion of red blood cells, initiated treatment with oral clarithro mycin and reinforced the rewarming of the extremities with good re - sults (Hb 9.6 g/dL). Regarding serological investigation, the results revealed a positive IgM for Mycoplasma pneumoniae ; positive Direct Antiglobulin Test with specificity for CD3, and positive Donath Land steiner test, leading to the diagnosis. This case report represents a syndrome that even though rare, is important to bear in mind when facing a case of severe haemolysis. Despite the favorable outcome, additional follow-up must be placed in outpatient setting, with periodic clinical and laboratorial revalua -tions.","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"31 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115610308","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-11-08DOI: 10.24966/hbtd-2999/100023
G. Monohan
{"title":"Perioperative And Postoperative Management In Patients With Alpha-2-Antiplasmin Deficiency: A Case Study Of 67-Year-Old Male Undergoing Operative Distal Femur Repair","authors":"G. Monohan","doi":"10.24966/hbtd-2999/100023","DOIUrl":"https://doi.org/10.24966/hbtd-2999/100023","url":null,"abstract":"","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"2 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133487273","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-11-08DOI: 10.24966/hbtd-2999/100022
M. Lugos
{"title":"Screening For Anaemia At Different Phases Of The Menstrual Cycle Among Female Students In A Nigerian University","authors":"M. Lugos","doi":"10.24966/hbtd-2999/100022","DOIUrl":"https://doi.org/10.24966/hbtd-2999/100022","url":null,"abstract":"","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"1222 ","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"113994804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-07-24DOI: 10.24966/hbtd-2999/100019
R. Williams-Hooker
Sickle Cell Disease (SCD) is a genetic disorder caused by a Hemoglobin gene mutation (HbS), which produces abnormal sickle he- moglobin that causes Red Blood Cells (RBC), to form rigid, sickle or holly-leaf-shaped cells rather than the typical round and flexible shape. These RBC’s are not able to pass through the vessels so less oxygen is transported to tissues, leading to vaso-occlusive pain cri - ses, organ damage, stroke, chronic anemia, decreased quality of life and early death. Nutritional concerns for children with SCD include weight loss and poor growth, delayed puberty, vitamin and mineral deficiencies and fluid imbalance. Several studies have shown that dietary intake falls below recommendations in SCD patients and that plasma levels of certain key vitamins, including vitamin E, are lower than reference values. Children with SCD have low to body water, which could be related, in part, to decreased intake. This study ex - plored vitamin E plasma status, vitamin E and fluid intake, and Total Body Water (TBW) status to determine if any of these variables were low. Each variable was also compared to pain scores to see if there was any correlation. Thirty African American children with SCD (15 female, mean age 12.03) participated in this study. Results showed that patients had decreased vitamin E and fluid intake, decreased corrected plasma vitamin E, and TBW below normal, however none of these factors was associated with pain medications. Further research needs to be conducted with a greater sample size and more pain variables.
{"title":"Vitamin E Status and Total Body Water in Children and Adolescents with Sickle Cell Anemia","authors":"R. Williams-Hooker","doi":"10.24966/hbtd-2999/100019","DOIUrl":"https://doi.org/10.24966/hbtd-2999/100019","url":null,"abstract":"Sickle Cell Disease (SCD) is a genetic disorder caused by a Hemoglobin gene mutation (HbS), which produces abnormal sickle he- moglobin that causes Red Blood Cells (RBC), to form rigid, sickle or holly-leaf-shaped cells rather than the typical round and flexible shape. These RBC’s are not able to pass through the vessels so less oxygen is transported to tissues, leading to vaso-occlusive pain cri - ses, organ damage, stroke, chronic anemia, decreased quality of life and early death. Nutritional concerns for children with SCD include weight loss and poor growth, delayed puberty, vitamin and mineral deficiencies and fluid imbalance. Several studies have shown that dietary intake falls below recommendations in SCD patients and that plasma levels of certain key vitamins, including vitamin E, are lower than reference values. Children with SCD have low to body water, which could be related, in part, to decreased intake. This study ex - plored vitamin E plasma status, vitamin E and fluid intake, and Total Body Water (TBW) status to determine if any of these variables were low. Each variable was also compared to pain scores to see if there was any correlation. Thirty African American children with SCD (15 female, mean age 12.03) participated in this study. Results showed that patients had decreased vitamin E and fluid intake, decreased corrected plasma vitamin E, and TBW below normal, however none of these factors was associated with pain medications. Further research needs to be conducted with a greater sample size and more pain variables.","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"17 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129665888","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-07-24DOI: 10.24966/HBTD-2999/100017
A. Zulfiqar
{"title":"Idiopathic Thrombocytopenic Purpura (ITP) in the Elderly Over 75 Years: About a Rare Series in the Very Elderly Subjects","authors":"A. Zulfiqar","doi":"10.24966/HBTD-2999/100017","DOIUrl":"https://doi.org/10.24966/HBTD-2999/100017","url":null,"abstract":"","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131277995","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-07-24DOI: 10.24966/HBTD-2999/100020
A. Momot
{"title":"Effects of Low-Molecular Heparin on Pregnant Women with Factor V Mutation (GA Genotype)","authors":"A. Momot","doi":"10.24966/HBTD-2999/100020","DOIUrl":"https://doi.org/10.24966/HBTD-2999/100020","url":null,"abstract":"","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"48 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124625461","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-07-24DOI: 10.24966/HBTD-2999/100018
M. M. Fresen
A Abstract Patients scheduled for high-dose chemotherapy who fail to mobilize a sufficient number of hematopoietic stem cells have a poor prognosis. Since 2008 the CXCR4-inhibitor plerixafor is available to improve stem cell collection and to reduce the number of failed mo -bilizers. The of Hodgkin Lymphoma (HD) and Multiple Myeloma (MM) was evaluated (group A). All patients received G-CSF with or without chemotherapy for mobilization. This group was matched by age, sex and diagnosis to 47 proven poor mobilizers receiving plerixafor (group B). In group A, 92.9% diagnosed with NHL and all patients diagnosed with MM and HD gathered more than 2.0 × 10^6 CD34+ cells/kg BW. In group B, 64.3% of the NHL patients, 88.2% of the patients diagnosed with MM and all patients with HD were able to collect the defined minimum of CD34+ cells. In total, 74.5% of poor mobilizing patients who received plerixafor gathered more than 2.0 × 10^6/kg BW CD34+ cells. Transplanted cells engrafted in both cohorts; how- ever, in NHL and MM patients, engraftment of white blood cells and platelets were significant earlier in group A than in group B. In conclusion, only 4.3% of patients failed first mobilization at -tempt. For these limited number of patients plerixafor is a valuable additive.
{"title":"Stem Cell Mobilization with and without Plerixafor: A Comparative Analysis","authors":"M. M. Fresen","doi":"10.24966/HBTD-2999/100018","DOIUrl":"https://doi.org/10.24966/HBTD-2999/100018","url":null,"abstract":"A Abstract Patients scheduled for high-dose chemotherapy who fail to mobilize a sufficient number of hematopoietic stem cells have a poor prognosis. Since 2008 the CXCR4-inhibitor plerixafor is available to improve stem cell collection and to reduce the number of failed mo -bilizers. The of Hodgkin Lymphoma (HD) and Multiple Myeloma (MM) was evaluated (group A). All patients received G-CSF with or without chemotherapy for mobilization. This group was matched by age, sex and diagnosis to 47 proven poor mobilizers receiving plerixafor (group B). In group A, 92.9% diagnosed with NHL and all patients diagnosed with MM and HD gathered more than 2.0 × 10^6 CD34+ cells/kg BW. In group B, 64.3% of the NHL patients, 88.2% of the patients diagnosed with MM and all patients with HD were able to collect the defined minimum of CD34+ cells. In total, 74.5% of poor mobilizing patients who received plerixafor gathered more than 2.0 × 10^6/kg BW CD34+ cells. Transplanted cells engrafted in both cohorts; how- ever, in NHL and MM patients, engraftment of white blood cells and platelets were significant earlier in group A than in group B. In conclusion, only 4.3% of patients failed first mobilization at -tempt. For these limited number of patients plerixafor is a valuable additive.","PeriodicalId":236918,"journal":{"name":"Hematology, Blood Transfusion and Disorders","volume":"61 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133954274","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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