A 68-year-old male with a past medical history of inflammatory bowel disease, alcoholism, smoking (cigars) for over 30 years, and COPD presented to the emergency department with shortness of breath, dizziness, poor appetite, and progressive weight loss of 30 lbs over a six-month period. He attributed his poor oral intake due to his dental extraction one month prior. The physical examination showed diffuse skin ecchymosis otherwise unremarkable at the time. Significant laboratory findings showed hemoglobin 12.5g/dl, platelets of 17,000 k/ul, white blood count 21,000 k/ul, potassium 2.6 mmol/L, lactic acid 10.9 mmol/L, lactate dehydrogenase 8,256 u/l and a negative flow cytometry. Peripheral smear displayed schistocytes without clumping. Blood cultures were positive for staphylococcus aureus in two culture bottles. Iron studies, b12, folate were all normal. He was negative for hepatitis, cytomegalovirus, Lyme disease, babesia, and HIV. The patient's platelet count was 402 k/ul approximately two years ago.
Computerized tomography (CT) scan demonstrated right-sided aspiration pneumonia and necrotic lymph nodes in the mediastinum. Induced sputum testing was negative three times for acid-fast staining in order to rule out tuberculosis. Echocardiogram was normal. He was treated with intravenous vancomycin and piperacillin-tazobactam but his condition began to deteriorate rapidly with new-onset atrial fibrillation, encephalopathy, decline in hemoglobin with melena, and worsening respiratory distress now requiring non-invasive positive airway pressure support. Due to his poor respiratory status, a bronchoscopy with biopsy could not be performed. The patient began treatment for TTP. He was transferred to the ICU for plasmapheresis with transfusions of platelets and administration of high dose steroids. His hemoglobin dropped to as low as 4.9 g/dl requiring multiple blood transfusions to maintain hemodynamic stability. The platelets continued to range between 5,000 u/l and 26,000 u/l. A bone marrow biopsy was done and intravenous immunoglobulin was administered. CD 25, natural killer cells, ADAMS13, and antiplatelet antibody were all normal.
Bone marrow biopsy results were positive for e-cadherin, cytokeratin CAM 5.2, CD117, and CD56 consistent with small cell infiltration of the bone marrow. He was started on imatinib, but at this point, he was no longer a candidate for chemotherapy. On hospital day 12, he became hypotensive resulting in multi-organ failure requiring high doses of norepinephrine. Later during the night, he began to exsanguinate from all of his orificesrequiring more blood transfusions. Laboratory results at this point showed an LDH of 14,000 u/l, Bun 109 mg/dl, Creatinine 3.1 mg/dl, PT 54 H, INR 5.83H, aPTT 42.6 H, AST 1597 u/l, and ALT 341u/l. The patient expired 15 days after presentation.