November Case 2021
A 49-year-old man presented with abdominal pain that progressed to generalized edema, ascites, pleural effusion, and acute renal failure, with anemia and thrombocytopenia. He was started on hemodialysis and underwent renal biopsy, which showed thrombotic microangiopathy. An autoimmune work-up was initiated while he was treated with steroids and plasmapheresis due to concern for thrombotic thrombocytopenic purpura (ADAMTS13 found to be normal). The autoimmune work-up was negative. He then developed respiratory failure and was intubated. Further work-up demonstrated polymicrobial pneumonia, CMV viremia, and VRE bacteremia. He was treated with broad-spectrum antibiotics, antimycotics, and steroids. Imaging showed hepatosplenomegaly, minimal periaortic lymphadenopathy and slowly resolving pneumonia. After a prolonged hospital course, which included tracheostomy and ventilator dependence, he was weaned from the ventilator and from dialysis and discharged.
He was later re-admitted for progressive shortness of breath, pleuritic chest pain, and abdominal pain. Chest x-ray showed bibasilar pulmonary infiltrates. A chest CT angiogram ruled out a pulmonary embolism and identified a multiloculated pleural effusion. Blood and pleural fluid cultures were obtained, and empiric antibiotic therapy was initiated for presumed community acquired pneumonia. Imaging also showed a new mediastinal mass with prominent adjacent lymph nodes, numerous prominent bilateral axillary lymph nodes, and splenomegaly was identified. Laboratory testing showed: WBC 14k cells/μL, Hgb 13g/dL, Plt 145k/μL, Cr 1.3mg/dL. Cultures were followed and found to be negative.
His respiratory symptoms improved with antibiotic therapy. However, imaging findings were concerning for an underlying lymphoproliferative disorder. A CT-guided biopsy of the mediastinal mass was non-diagnostic. A bone marrow biopsy showed a hypercellular marrow with severe myelofibrosis and no significant dysplasia or increase in blasts; JAK2, CALR, and MPL testing was negative. He then developed acute renal failure with markedly increased creatinine and potassium associated with worsening generalized edema. Repeat laboratory testing showed: WBC 9k cells/μL, Hgb 9g/dL, Plt 90k/μL, Cr 3mg/dL, IL-6 22pg/mL (normal range: 3-14pg/dL). HIV and HHV8 testing were negative.
Axillary lymph node biopsies were obtained.
The low power view shows lymph node tissue with abnormal follicles, including partial hyalinization, regression of germinal centers, and concentric mantle zone lymphocytes showing "onion skinning" appearance. Several follicles show prominent penetrating venules. No Hodgkin-Reed-Sternberg cells or LP cells were identified.
Immunohistochemical staining shows a polytypic plasmacytosis with no increase in IgG4+ plasma cells. HHV8 staining is negative.
Discussion of Diagnosis
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