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Undiagnosed Hematopoietic Neoplasms Discovered at Autopsy

Undiagnosed Hematopoietic Neoplasms Discovered at Autopsy

Case 2


A 67-year-old African American man with a history of a T-cell lymphoma diagnosed 10 years prior within the iliac lymph nodes, a retroperitoneal mass (following autologous bone marrow transplant), prostate cancer (following irradiation), and recently diagnosed hepatic cirrhosis and ascites came to the emergency department with bloody stools, altered mental status, and generalized weakness. Admission laboratory values included serum urea nitrogen, 144 mg/dL; creatinine, 6.6 mg/dL; alkaline phosphatase, 146 U/L; AST, 48 U/L; ALT, 43 U/L; and anion gap, 17. Lactate measured 2.0 mmol/L. Computed tomography (CT) found a shrunken liver with a slightly nodular surface contour, compatible with cirrhosis; no focal hepatic mass was noted. Esophagogastroduodenoscopy to detect the source of the gastrointestinal bleed was unable to be performed due to the patient's unstable state. Echocardiogram found a large pericardial effusion, and subsequent pericardiocentesis removed 1,100 mL of bloody fluid. Flow cytometry of the pericardial effusion revealed a monoclonal B-cell population. He became increasingly hemodynamically unstable and developed disseminated intravascular coagulopathy requiring multiple transfusions and vasopressors. Lactate dehydrogenase was 258 U/L. Lactic acid increased to 10.1 mmol/L and pH was 7.26 with an anion gap of 17. The ALT was 640 U/L, AST was 1,794 U/L, and total bilirubin was 4.0 g/dL. He eventually died under comfort care measures.

Autopsy found two areas of mucosal irregularity (1.5–2.0 cm) between the body and the antrum of the stomach. The pleural cavities contained 700 mL and 750 mL of clear serous fluid in the right and left cavities, respectively. The liver, which was diffusely cirrhotic and indurated, weighed 1,150 g. The pericardium and epicardium of the heart were covered by a fibrinous exudate. Microscopically, the gastric biopsy specimen and sections from the mucosal irregularities showed fragments of gastric mucosa with a dense lymphoid infiltrate composed of large atypical cells. There was brisk mitotic activity with some apoptotic debris. The lymphoid cells were immunohistochemically positive for CD20, CD79a, MUM1, and CD22 and negative for CD10 and BCL6, consistent with a diffuse large B-cell lymphoma, activated B-cell type. Tumor cells were also identified in the spleen and the pericardium. The bone marrow was negative. There was no evidence of residual T-cell lymphoma or prostatic adenocarcinoma.

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