Prolymphocytoid transformation of a splenic B-cell lymphoma presenting as a spontaneous splenic rupture.
Abu Sayf A, Nakhle A, Tatem G, and Polevoy R. Prolymphocytoid transformation of a splenic B-cell lymphoma presenting as a spontaneous splenic rupture. Chest 2016; 150(4):258A.
Spontaneous splenic rupture as an initial presentation of splenic B-cell lymphoma is a very rare clinical entity. We present a case of prolymphocytoid transformation with catastrophic outcome. CASE PRESENTATION: A 71 year old woman was hospitalized for a COPD exacerbation for 4 days and 1 day prior to discharge suddenly developed signs of hypotension, abdominal distention and altered mental status. A CT of the chest, abdomen and pelvis showed acute splenic rupture with ascites and portal lymphadenopathy. Patient underwent emergent splenectomy. WBC was 86K with 40 % lymphocytes and peripheral smear showed 60 % prolymphocytes. Bone marrow biopsy confirmed these findings. Immunophenotyping rulled out similar hematologic malignancies such as mantle cell lymphoma and chronic lymphocytic lymphoma, in addition patient did not meet the criteria for the diagnosis of B-cell prolymphocytic leukemia. Splenic pathology was consistent with prolymphocytoid transformation of splenic B-cell lymphoma. Chemotherapy was started; however, her course was complicated with tumor lysis syndrome and subsequent multi organ failure which resulted in her expiring 2 weeks later. DISCUSSION: Acute abdomen secondary to splenic rupture in the absence of trauma or procedures is exceedingly rare. Splenic B-cell lymphoma is a very rare entity of B cell lymphoma and was previously undiagnosed in this patient until she developed prolymphocytoid transformation. In a case series of 613 patients with spontaneous splenic rupture reported by Aubrey and Sowers in 2012, 28 patients out of 84 cases of hematologic malignancies were diagnosed with non-hodgkin's lymphoma; yet non of them were classified as Prolymphocytoid transformation. Hoedhn and colleuges repoted 4 cases with such a diagnosis in 2012 which all were caracterized with massive splenomaegaly. CONCLUSIONS: Our case carries an unusual initial presentation of Prolymphocytoid transformation of a splenic B-cell lymphoma with a devastating outcome.