J Korean Med Sci.  2011 Jun;26(6):824-828. 10.3346/jkms.2011.26.6.824.

Waldenstrom Macroglobulinemia with CD5+ Expression Presented as Cryoglobulinemic Glomerulonephropathy: A Case Report

• Kim YL
• Gong SJ
• Hwang YH
• Joo JE
• Cho YU
• Lee JA
• Sung SA
• Lee SY
• Kim NY

• KMID: 1785971
• DOI: http://doi.org/10.3346/jkms.2011.26.6.824

Abstract

Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.

Keyword

Waldenstrom Macroglobulinemia; CD5+ Lymphoplasmacytic Lymphoma; Cryoglobulinemic Glomerulonephritis

MeSH Terms

Antigens, CD5/*metabolism
Antineoplastic Agents/therapeutic use
B-Lymphocytes/immunology/metabolism
Bone Marrow/pathology
Cryoglobulinemia/diagnosis
Cyclophosphamide/therapeutic use
Diagnosis, Differential
Doxorubicin/therapeutic use
Drug Therapy, Combination
Female
Glomerulonephritis/*diagnosis/pathology
Humans
Kidney/pathology
Middle Aged
Paraproteinemias/diagnosis
Prednisolone/therapeutic use
Vincristine/therapeutic use
Waldenstrom Macroglobulinemia/*diagnosis/drug therapy/pathology

Figure

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  Fig. 1 Renal biopsy. (A) Immunofluorescent microscopic study showed 2+ reaction for IgM. (B) On the electron microscopic (EM) findings (× 20,000), there are subendothelial (arrow) and mesangial electron dense deposits revealing microtubular structures (25 nm in average diameter).

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  Fig. 2 Serum immunofixation electrophoresis. (A) There is a slightly dense band with IgM, kappa antisera, suggestive of monoclonal gammopathy (B) After the treatment, a dense band with IgM was disappeared.

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  Fig. 3 Bone marrow biopsy. (A) Positive reaction in small lymphocytes with anti-CD5 antibody (immunohistochemical stain, × 400). (B) Positive reaction in small numbers of plasma cells with anti-cytoplasmic IgM antibody (immunohistochemical stain, × 400).

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  Fig. 4 Ophthalmoscopic examination revealed dilatation and tortuosity of the retinal veins.

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