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μ‐Chain Disease in a Case of Chronic Lymphocytic Leukaemia and Malignant Histiocytoma
Author(s) -
Jønsson Viggo,
Videbæk Aage,
Axelsen Nils H.,
Harboe Morten
Publication year - 1976
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1976.tb01140.x
Subject(s) - lymphocytosis , leukocytosis , medicine , proteinuria , chlorambucil , prednisone , bence jones protein , chronic lymphocytic leukemia , bone marrow , pathology , immunoglobulin light chain , leukemia , immunology , kidney , antibody , chemotherapy , cyclophosphamide
A 27‐year‐old woman with proteinuria and normal serum creatinine for 10 years developed chronic lymphocytic leukaemia (CLL) and after further 2 years during treatment with prednisone and chlorambucil a malignant histiocytoma appeared. Free μ‐chains were demonstrated in serum, but it is possible that μ‐chain disease (μ‐CD) and Bence‐Jones proteinuria had persisted unrecognized for 12 years, since vacuolated plasma cells were found when the primary bone marrow smear was re‐evaluated and no glomerular disorders were found. The clinical findings from the now 11 known cases of μ‐CD are reveiwed and the immunochemical findings are reported in part II (Axelsen et al 1976). The patient's mother died of acute leukaemia at the age of 24. The mother's sister has mammary carcinoma and several members of the mother's line apparently have increased tendency to infections. Of 27 family members, none exhibited immunoglobulin defects, but 10 members of the mother's line had B‐ as well as T‐lymphocytosis and 5 members had leukocytosis.