- Email: [email protected]
Prolymphocytoid Transformation of a Splenic B-Cell Lymphoma Presenting as a Spontaneous Splenic Rupture
Critical Care SESSION TITLE: Fellow Case Report Poster - Critical Care III SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM
Prolymphocytoid Transformation of a Splenic B-Cell Lymphoma Presenting as a Spontaneous Splenic Rupture Alaa Abu Sayf MD* Asaad Nakhle MD Geneva Tatem MD; and Rima Polevoy MD Henry Ford Hospital, Detroit, MI INTRODUCTION: 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.
CRITICAL CARE
CONCLUSIONS: Our case carries an unusual initial presentation of Prolymphocytoid transformation of a splenic B-cell lymphoma with a devastating outcome. Reference #1: Hoehn D, Splenic B-cell lymphomas with more than 55% prolymphocytes in blood: evidence for prolymphocytoid transformation. Epub 2012 Apr 18. Hum Pathol. 2012 Nov;43(11):1828-38. DISCLOSURE: The following authors have nothing to disclose: Alaa Abu Sayf, Asaad Nakhle, Geneva Tatem, Rima Polevoy No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.271
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
258A
[
150#4S CHEST OCTOBER 2016
]
Prolymphocytoid Transformation of a Splenic B-Cell Lymphoma Presenting as a Spontaneous Splenic Rupture Alaa Abu Sayf MD* Asaad Nakhle MD Geneva Tatem MD; and Rima Polevoy MD Henry Ford Hospital, Detroit, MI INTRODUCTION: 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.
CRITICAL CARE
CONCLUSIONS: Our case carries an unusual initial presentation of Prolymphocytoid transformation of a splenic B-cell lymphoma with a devastating outcome. Reference #1: Hoehn D, Splenic B-cell lymphomas with more than 55% prolymphocytes in blood: evidence for prolymphocytoid transformation. Epub 2012 Apr 18. Hum Pathol. 2012 Nov;43(11):1828-38. DISCLOSURE: The following authors have nothing to disclose: Alaa Abu Sayf, Asaad Nakhle, Geneva Tatem, Rima Polevoy No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.271
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
258A
[
150#4S CHEST OCTOBER 2016
]