Case Reports in Hematology最新文献

Central nervous system (CNS) involvement in multiple myeloma (MM) (MM-CNS) in the form of leptomeningeal myelomatosis or brain parenchyma plasmacytoma is rare, causing challenges in clinical diagnosis and treatment. We would like to report a case of leptomeningeal myelomatosis and illustrated the challeges. A 61-year-old man was diagnosed with MM with left paravertebral plasmacytoma, R-ISS II with high suspicion of double-hit MM, either biallelic aberrancy of TP53 or del(17p) and IGH aberrancy depending on the definition chosen, treated with lenalidomide-bortezomib-dexamethasone and local radiotherapy, later developed systemic relapse and progression to MM-CNS in the form of leptomeningeal myelomatosis. A modified CNS-based treatment not reported before, consisting of daratumumab, pomalidomide, vincristine, procarbazine, and dexamethasone, brought a rapid clinical improvement and warrants a further study. Incorporation of intrathecal thiotepa into the regimen would likely increase the efficacy.

Angioimmunoblastic T-cell lymphoma (AITL) accounts for 18.5% of all peripheral T-cell lymphomas. There is still no gold standard chemotherapy for treating newly diagnosed AITL. This case describes the use of bortezomib in newly diagnosed AITL. A 53-year-old man with no previous illness presented with erythema and swelling in the left neck. A diagnosis of AITL was made based on the results of lymph node biopsies. AITL progression led the patient to a severely deteriorated general condition. Bortezomib was thus administered, which resulted in a reduction in lymphadenopathies, the disappearance of tumor fever, and a decrease in serum lactate dehydrogenase levels. Subsequently, the patient's general condition gradually improved. Despite the patient's poor condition, bortezomib was well tolerated. After bortezomib administration, the patient did not require chemotherapy for approximately 10 months. The present case indicates that bortezomib is a possible treatment option for patients with AITL.

Coronavirus disease 2019 (COVID-19) is an acute complex systemic disorder caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).While SARS-CoV-2 is known to cause significant pulmonary disease, various extrapulmonary manifestations of COVID-19 have also been reported. Growing evidence suggests that COVID-19 is associated with coagulopathy leading to micro and macrovascular complications. Although in patients with COVID-19, venous thromboembolic events are more frequent, arterial thrombosis also occurs at an increased rate. These often lead to acute life-threatening ischemia, which requires urgent diagnosis and treatment. We present case reports of two patients with an abnormal thrombus formation in the thoracic aorta who recently overcame COVID-19, which led to systemic embolism and splenic infarction. Ambulatory oral factor Xa inhibitor therapy led to aortic thrombosis resolution in both patients.

Sickle cell disease (SCD) exists on a phenotypic spectrum with variable genetic expressivity, making it difficult to assess an individual patient's risk of complications at any particular point in time. Current and emerging SCD treatments, including CRISPR-based gene editing, result in a variable proportion of affected red blood cells (RBCs) still vulnerable to sickling. Clinical serological indicators of disease such as hemoglobin, indirect bilirubin, and reticulocyte count and clinical metrics including number of emergency department visits and hospitalizations over time often fall short in their ability to objectively quantify ischemic disease activity and efficacy of treatments. Clearly, better clinical biomarkers are needed. The rapidly developing field of oculomics leverages the transparent nature of the ocular tissue to directly study the retinal microvasculature in order to characterize the status of systemic diseases. In this case report, we demonstrate the ability of optical coherence tomography angiography (OCT-A) to detect and measure micro-occlusive events within the retinal capillary bed before and after RBC exchange transfusion and following CRISPR-based gene editing, as an indicator of systemic ischemic disease activity and measure of treatment efficacy. The implications of these findings are discussed.

Dengue is a mosquito-borne viral illness common in tropical and subtropical countries but very rare in the United States. Patients infected with dengue often present with thrombocytopenia. In the setting of dengue, platelet transfusions as a treatment for thrombocytopenia have no clear benefits in reduction of severe bleeding or improvement of the platelet count. Here, we present a case of a traveler infected with dengue virus and discuss the approach to treat thrombocytopenia.

We report a patient with hereditary erythrocytosis who underwent a therapeutic phlebotomy and had a post-phlebotomy hematocrit that was higher than the pre-phlebotomy hematocrit. We could not discern a reason for this hematocrit increase after phlebotomy. Instead of performing another phlebotomy, we performed an automated red cell depletion via an apheresis instrument. This procedure is essentially a red cell exchange, but 5% albumin is used as the replacement fluid instead of red blood cells. The patient's hematocrit decreased from 80% to 39% after three consecutive daily red cell depletion procedures. We share our experience to report the unusual finding of a patient's hematocrit that increased with phlebotomy and to raise awareness of the red cell depletion procedure.

Characterized by an aggressive course with a poor overall survival due to treatment refractoriness, plasmablastic lymphoma (PBL) is a rare variant of diffuse large cell B cell lymphoma. Gorham's lymphangiomatosis or Gorham-Stout disease (GSD) is a rare skeletal condition of unknown etiology characterized by progressive bone loss and nonmalignant proliferation of vascular and lymphatic channels within the affected bone. Neither disease has a standard of care. We present a 23-year-old HIV-negative woman with GSD, managed medically with octreotide and sirolimus, who developed PBL. After progressing on V-EPOCH (bortezomib, etoposide, vincristine, cyclophosphamide, doxorubicin, and prednisone), she was treated with daratumumab, lenalidomide, and dexamethasone (DRD) therapy and achieved complete remission after two cycles with progression after eight cycles. This is a report of treatment of PBL with DRD therapy. Clinical investigations of the DRD regimen in PBL in conjunction with other agents to improve both depth and durability of response are warranted.

Several vaccines have been developed and are being administered against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Common side effects include fever, chills, headache, myalgia, and soreness at the injection site. However, some rare adverse effects have also been reported. We present a case of induced thrombocytopenia presenting with petechiae and mucosal bleeding which developed as an adverse response after first-dose administration of the Moderna COVID-19 vaccine.

Chronic myeloid leukemia (CML) is a clonal hematopoietic stem cell disorder, characterized by reciprocal translocation t(9,22) (q34; q11), leading to increased myeloid proliferation. Most cases are diagnosed in the chronic phase (CP). However, a minority of cases can be present in the blastic phase (BP). In most patients with CML-BP, the blasts have a myeloid phenotype, however, in 20-30% of cases, the blasts have a lymphoid phenotype, mostly a B-cell phenotype. It is challenging to differentiate CML B-lymphoblastic phase (CML-BLP) from Ph + primary B-acute lymphoblastic leukemia (B-ALL) especially when the CML-BLP is the initial presentation of the disease, which is uncommon. We report here an unusual case of CML-BLP as an initial presentation of the disease without typical CML morphological findings. This case demonstrates diagnostic challenges and emphasizes the importance of an integrated approach using morphology, multiparametric flow cytometry, cytogenetic studies, and molecular studies to render an accurate diagnosis.