CNS intravascular large cell lymphoma in a patient with autoimmune hemolytic anemia

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‐oc...

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Bibliographic Details
Published in:Neuropathology 2015-04, Vol.35 (2), p.170-174
Main Authors: Alexandrescu, Sanda, Orengo, James P., Toossi, Shahed, Perry, Arie, Treseler, Patrick, Hess, Christopher, Margeta, Marta
Format: Article
Language:English
Subjects:
Adult
Anemia
Anemia, Hemolytic, Autoimmune - complications
Anemia, Hemolytic, Autoimmune - pathology
autoimmune hemolytic anemia
Brain Neoplasms - complications
Brain Neoplasms - pathology
Drug therapy
Fatal Outcome
Female
Humans
intravascular lymphoma
leukoencephalopathy
Lymphoma
Lymphoma, Large B-Cell, Diffuse - complications
Lymphoma, Large B-Cell, Diffuse - pathology
Mortality
Vascular Neoplasms - complications
Vascular Neoplasms - pathology
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Summary: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.
ISSN:0919-6544
1440-1789
DOI:10.1111/neup.12175