23 May 2008 – Case of the Week #120
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We thank Dr. Ankur Sangoi, Stanford University, Stanford, California (USA) for contributing this case. To contribute a Case of the Week, email NatPernick@Hotmail.com with the clinical history, your diagnosis and microscopic images in JPG, GIF or TIFF format (send as attachments, we will shrink if necessary). Please include any other images (gross, immunostains, etc.) that may be helpful or interesting. We will write the discussion (unless you want to), list you as the contributor, and send you $35 (US dollars) for your time after we send out the case. Please only send cases with high quality images and a diagnosis that is somewhat unusual (or a case with unusual features).
Case of the Week #120
An 88 year old woman presented with a cough. A CBC (complete blood count) and peripheral smear were obtained.
Based on these results, flow cytometry was performed on the peripheral blood:
What is your diagnosis?
B cell prolymphocytic leukemia
The peripheral smear shows a relatively monomorphic population of large lymphocytes with scant basophilic agranular cytoplasm, round / polygonal nuclei with occasional grooves and large, prominent nucleoli.
Flow cytometry shows a monomorphic B
cell population which accounts for the majority of lymphocytes, and which expresses
CD19, CD20 and CD5, with bright monotypic kappa light chain restriction and
dim/partial expression of CD22, CD23 and FMC7. These findings are diagnostic
of B cell prolymphocytic leukemia (B-PLL).
B-PLL is a rare chronic lymphoid leukemia that typically affects elderly men, who present with a peripheral blood lymphocytosis > 100,000, marked splenomegaly and pancytopenia. By definition, prolymphocytes comprise more than 55% of the leukemic infiltrate. The prolymphocytes are found in the splenic red and white pulp, as well as the peripheral blood. Most (80%) cases are B cell phenotype.
The differential diagnosis includes prolymphocytic transformation of CLL (patient has prior history of CLL or presence of CLL cells), as well as the blastic variant of mantle cell lymphoma, which occasionally presents with more than 55% prolymphocytes, but is positive for t(11;14) (AJCP 2001;115:571, AJCP 2002;117:246).
B-PLL patients have a better prognosis than T-PLL patients, although the disease is still considered incurable. Treatment, for symptomatic patients only, consists of monoclonal antibodies (Ann Hematol 2004;83:319), high dose chemotherapy followed by stem cell transplantation, or fludarabine-like drugs (Med Sci Monit 2007;13:RA69).
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