Intravascular anaplastic large cell lymphoma may be difficult to diagnose since patients may present in various ways depending on the organs involved and since there is no lymphadenopathy. Diagnosis often requires a high index of suspicion and the identification of intravascular lymphoma cells in needle biopsy.


The lymphoma may be composed of B or T cells.


Clinical findings:

(1) fever of unknown origin (FUO)

(2) unexplained fatigue

(3) mental status changes, dementia

(4) focal neurologic signs or nonlocalizing neurologic deficits

(5) polyneuropathy

(6) skin rash (plaques or subcutaneous nodules)

(7) cough and/or dyspnea and/or asthma

(8) urinary retention

(9) nephrotic syndrome

(10) renal failure

(11) hypertension

(12) unexplained weight loss

(13) fulminant hepatic failure

(14) rapidly progressing disease resulting in death


Laboratory findings:

(1) hemolytic anemia with positive direct antiglobulin test

(2) leukopenia

(3) thrombocytopenia, including DIC

(4) elevated liver function tests

(5) polyclonal gammopathy

(6) metabolic acidosis


Identification of intravascular lymphoma cells:

(1) bone marrow biopsy

(2) renal biopsy

(3) liver biopsy

(4) skin biopsy

(5) prostate biopsy

(6) brain biopsy

(7) other tissue removed at surgery


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