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A rare case of pancytopenia secondary to copper deficiency

A rare case of pancytopenia secondary to copper deficiency

Dr Kapil Saxena at the University of California, San Francisco and colleagues have reported a rare case of pancytopenia secondary to copper deficiency that has appeared in the JAMA Clinical Challenge.

Hypocupremia, or copper deficiency, is a rare and underrecognized cause of bone marrow dysplasia. Most cases of copper deficiency in adults occurred historically in patients receiving total parenteral hyperalimentation or total parental nutrition.

According to history, a 54-year-old man presented for evaluation of new-onset pancytopenia. He had a distant history of viral myocarditis and was taking lisinopril, carvedilol, furosemide, aspirin, and a multivitamin. No new medications had been started in the past year. Twelve years prior, he underwent uncomplicated Roux-en-Y gastric bypass (RYGB) surgery. Review of systems was notable for fatigue and negative for fever, night sweats, weight loss, dyspnea, or abnormal bleeding. Examination revealed the temperature of 36.7°C; heart rate, 91/min; blood pressure, 120/70 mm Hg; and body mass index, 20.1 (calculated as weight in kilograms divided by height in meters squared). He was well-appearing with conjunctival pallor but no petechiae, no hepatosplenomegaly, and a normal neurologic examination.

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Courtesy JAMA Clinical Challenge.

His Laboratory evaluation showed a white blood cell count of 1.7 × 103/μL with an absolute neutrophil count of 0.17 × 103/μL; haemoglobin level, 7 g/dL with a mean corpuscular volume of 103 fL (reticulocyte index, 0.94); and platelet count, 116 × 103/μL. Results of a complete blood cell count from 8 months prior were normal. Results of serum protein electrophoresis were normal, as were levels of lactate dehydrogenase, ferritin, folate, vitamin B12, and thyroid-stimulating hormone. A computed tomography (CT) scan of the abdomen showed no lymphadenopathy, masses, or hepatosplenomegaly. A peripheral blood smear showed occasional pseudo-Pelger-Huet cells and rare teardrop cells. Histopathologic examination of bone marrow aspirate revealed a normocellular marrow and dyserythropoiesis with vacuolized and dysplastic erythroid precursors (Figure, panel A), left-shifted granulocytic maturation with vacuolized granulocytic precursors, ring sideroblasts (Figure, panel B), and no increase in blasts. Cytogenetic studies from the bone marrow did not reveal any abnormalities.

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Diagnosed as a case of pancytopenia secondary to copper deficiency, Supplementation was initiated with oral copper gluconate (4 mg 3 times daily). Within 2 months, serum copper level normalized and blood counts improved (hemoglobin, 13.2 g/dL; white blood cell count, 8.5 × 103/μL; absolute neutrophil count, 5.6 × 103/mL; and platelet count, 202 × 103/mL). The copper gluconate dosage was subsequently reduced and maintained at 2 mg daily without recurrence of cytopenias.

The key to the correct diagnosis, in this case, is the patient’s history of gastric bypass surgery, a risk factor for micronutrient deficiencies including copper deficiency. Copper deficiency rarely develops in patients without a history of malabsorption, as the average intake of 1 to 1.6 mg/d typically exceeds the estimated requirement of 0.9 mg/d.1,2 However, RYGB surgery and other bariatric procedures including partial gastrectomy are underrecognized causes of copper deficiency.
Other conditions associated with copper deficiency include celiac disease and excessive zinc ingestion. Copper absorption in celiac disease is directly impaired via malabsorption due to small intestinal inflammation.

Patients with prolonged copper deficiency can develop both neurologic and hematologic abnormalities. Neurologic manifestations of copper deficiency include myeloneuropathy changes that present as an unsteady gait, distal muscle weakness, or sensory deficits in the extremities.

For more details click on the link: doi:10.1001/jama.2018.21899

Source: self

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