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CNS intravascular large cell lymphoma in a patient with autoimmune hemolytic anemia
Author(s) -
Alexandrescu Sanda,
Orengo James P.,
Toossi Shahed,
Perry Arie,
Treseler Patrick,
Hess Christopher,
Margeta Marta
Publication year - 2015
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/neup.12175
Subject(s) - medicine , autopsy , autoimmune hemolytic anemia , intravascular large b cell lymphoma , leukoencephalopathy , thrombotic microangiopathy , hemolytic anemia , lymphoma , anemia , differential diagnosis , pathology , complication , microangiopathy , disease , pediatrics , diabetes mellitus , endocrinology
Intravascular large cell lymphoma ( IVLCL ) is a rare disease characterized by proliferation of malignant lymphocytes within the small blood vessel lumens. The association of IVLCL with autoimmune hemolytic anemia ( AIHA ) has been described in a single case report, but the true prevalence of this co‐occurrence is not known because of declining autopsy rates. Here, we report a case of a 41‐year‐old woman who carried a diagnosis of AIHA for 2 years, with repeated hemolytic episodes that were initially well controlled with immunomodulatory treatment. At her last presentation, the patient developed rapidly progressive neurologic symptoms and leukoencephalopathy on MRI; she died 4 weeks later with a clinical impression of thrombotic microangiopathy, a known complication of AIHA . At autopsy, the brain showed widespread platelet thrombi and intraparenchymal hemorrhages characteristic of this disorder. In addition, there was evidence of a clinically unsuspected IVLCL , most likely of B ‐cell lineage. This case illustrates a potential association between IVLCL and AIHA , highlights the need for broad differential diagnosis in cases with atypical disease presentation or progression, and underlines the importance of autopsy in establishing the full cause of morbidity and mortality.