A 5 6-year-old female presen ts For evaluation of malaise and a recent uninten tional 1 0-pound weight loss. Patien t reports no nigh t sweats or Fevers. but reports enlarged lymph nodes in her neck area. She also men tions several weeks of epistaxis and blurry vision. Physical exami nation con firms cervical lymphadenopathy and splenomegaly, and petech iae i nvolvi ng both lower extremi ties. Fundoscopic exam shows retinal hemorrhages and engorged retinal veins. Laboratory evaluation shows leukocytes of 1 2 . 700/pL hemoglobin of 1 0.8 g/dl, and platelet count of 1 78.000/JLL. Peri pheral slide is shown below.
Bone marrow biopsy is obtai ned and shows a hypercellular bone marrow with diffuse i nvolvemen t by small lymphocytes as shown below. Flow cytometry revealed positivity For CD 1 9 , CD20. and CD 1 38. with negative expression of CDS and CD2 3.
What is the most likely diagnosis?
- Follicular lymphoma (FL)
- Small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL)
- Lymphoplasmacytic lymphoma (LPL)
- Mantle cell lymphoma (MCL)
- Diffuse large B-cell lymphoma (DLBCL)
Medicine
C. Peripheral smear reveals the presence of circulating cells with plasmacytoid features. These cells resemble lymphocytes and are characterized by the presence of basophilic cytoplasm, ec-centric nucleus with perinuclear clearing, similar to plasma cells. Chromatin can be found in a typical clock-face pattern as seen above. The bone marrow is typically diffusely infiltrated by the LPL cells, and mast cells can also be seen (deeply basophilic cell above). LPL is an indolent small B-cell lymphoma, and the peripheral blood shows a leukemic phase in approximately 30% of cases. Waldenstrom macroglobulinemia (WM) is a specific subtype of LPL and is defined as LPL with bone marrow in-volvement associated with IgM monoclonal gammopathy and may present with symptoms related to hyperviscosity. Expression of typical plasma cell markers (CD 138) supports the diagnosis of LPL!WM. Lack of CDS and CD23 expression makes the diagnosis ofSLL/CLL and MCL less likely (Br J Haematol. 2001;1 1 5(3):575, Semin Oneal. 2003;30(2):110, J Clin Oneal. 2005;23(7):1564).
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