Secondary Hemophagocytic Syndrome Associated with Richter's Transformation in Chronic Lymphocytic Leukemia

نویسندگان

  • Nura El-Haj
  • Wilson I. Gonsalves
  • Vinay Gupta
  • Jacob P. Smeltzer
  • Sameer A. Parikh
  • Preet P. Singh
  • Naseema Gangat
چکیده

Hemophagocytic syndrome (HPS) is an extremely rare condition arising from the overactivation of one's own immune system. It results in excessive inflammation and tissue destruction. Prompt initiation of treatment is warranted in either scenario in order to decrease mortality. Most cases are triggered by infectious agents, malignancy, or drugs. We describe the first case of a CLL patient presenting with HPS due to acquisition of EBV-related large cell lymphoma in the setting of profound immunodeficiency.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Richter's syndrome: a novel presentation.

Chronic lymphocytic leukaemia with transformation into a large cell lymphoma (Richter's syndrome) is usually associated with the rapid demise of these already immunocompromised patients. There have been no previously recorded cases involving the nose. We report such a case and describe a successful treatment approach combining monoclonal antibody therapy with ao mini-allogeneic bone marrow tran...

متن کامل

Secondary CD5+ diffuse large B-cell lymphoma not associated with transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma (Richter syndrome).

Few cases of secondary CD5+ diffuse large B-cell lymphoma (DLBCL) that are not Richter syndrome have been reported previously. We report 9 cases of non-Richter syndrome secondary CD5+ DLBCL. Among 529 cases of DLBCL, 38 (7.2%) were CD5+ DLBCL, including 9 of secondary CD5+ DLBCL. Five cases gained CD5 expression during the clinical course of DLBCL (group 1). Three cases showed transformation fr...

متن کامل

Hodgkin lymphoma in a patient with chronic lymphocytic leukaemia--a rare presentation of Richter's transformation.

Richter's transformation of chronic lymphocytic leukaemia (CLL) to high-grade B-cell Non-Hodgkin lymphoma occurs in < 5% of CLL cases. Transformation of CLL to Hodgkin Lymphoma is a much rarer event and here we describe a patient who developed Richter's transformation into a Hodgkin Lymphoma presenting as rapidly progressive hepatosplenomegaly.

متن کامل

Hemophagocytic lymphohistiocytosis secondary to T-cell Acute Lymphoblastic Leukemia with membranous tonsillitis

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation, which is characterized by fever, hepatosplenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, and/or hypofibrinogenemia, and evidence of hemophagocytosis. Secondary HLH is often seen in adults and categorized based on autoimmune, infections-related, and malignancy-associated etiol...

متن کامل

Intravascular large B‐cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia

Intravascular large B-cell lymphoma (IVLBCL) is an aggressive non-Hodgkin's lymphoma which can present with B symptoms, rash, and neurological deterioration. Up to 10% of cases of IVLBCL are associated with other hematological neoplasms, including this extremely rare presentation of IVLBCL as Richter's transformation in chronic lymphocytic leukemia.

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:

دوره 2014  شماره 

صفحات  -

تاریخ انتشار 2014