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Adobe zii er 4.412/16/2023 ![]() ![]() Computed tomography (CT) scan revealed an unremarkable spleen with multiple enlarged periaortic and aortocaval lymph nodes, the largest measuring 1.6 × 1.3 cm. Fluorescence in situ hybridization demonstrated a deletion at 20q, and molecular studies were negative for BCR- ABL fusion and Janus kinase 2 ( JAK2) mutations, which further supported the MDS diagnosis. Bone marrow core biopsy and aspirate showed a hypercellular marrow with dysplastic myeloid and megakaryocytes lineage, consistent with MDS–refractory cytopenia with multilineage dysplasia. We report a case of NHS-EMH involving a periaortic lymph node in a 73-year-old African-American man, with a history of anemia and thrombocytopenia for approximately 1.5 years, who presented with unintended weight loss of 30 pounds during 6 months. To our knowledge, only 4 NHS-EMH cases have been reported in patients with myelodysplastic syndrome (MDS): 2 were cutaneous EMH and 2 involved the paraspinal epidural space. Most reported cases were associated with myeloproliferative neoplasms. Usual involved sites are the paravertebral region, retroperitoneum, and lymph nodes. Nonhepatosplenic extramedullary hematopoiesis (NHS-EMH) is rare. Therefore, the findings in our case indicate that megaloblastic anemia has to be considered in the differential diagnosis of microangiopathic anemia, particularly in patients with high risk for vitamin B 12 deficiency (autoimmune disease, gastrointestinal surgery, and dietary deficiency) and dramatic increase in serum lactate dehydrogenase. After 3 to 4 days of vitamin B 12 treatment, the patient had a significant improvement in pancytopenia. Additional blood studies showed dramatically increased lactate dehydrogenase at 7330 μ/L (reference range, 98–192 μ/L) and methylmalonic acid level at 9.58 μmol/L (reference range, 0.00–0.40 μmol/L) with decreased vitamin B 12 at 40 pg/mL (reference range, 180–914 pg/mL). A bone marrow biopsy showed a hypercellular marrow with predominant megaloblastic erythroid hyperplasia ( Figure 1, B through D). The initial differential diagnosis included thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and other causes of microangiopathic hemolytic anemia. The coagulation profile displayed elevated activated partial thromboplastin time (39.4 seconds) and prothrombin time (16.8 seconds) but fibrinogen level (353 mg/dL) within reference range. Prominent anisopoikilocytosis and frequent schistocytes were noted ( Figure 1, A). ![]() His peripheral blood revealed severe pancytopenia, including marked normocytic anemia (hemoglobin level, 7.0 g/dL mean corpuscular hemoglobin volume, 85.7 fL) and profound thrombocytopenia (platelet count, 34 × 10 9/L). ![]() A 21-year-old Asian man with a history of common variable immune deficiency presented with fatigue and shortness of breath on light exertion. We report a case of megaloblastic anemia with unusual presentations, closely resembling microangiopathic anemia. Symptomatic megaloblastic anemia typically shows characteristic findings in the peripheral blood with increased mean corpuscular volume, macroovalocytes, and hypersegmented neutrophils. ![]()
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