Four aged siblings with B cell chronic lymphocytic leukemia.

All four aged siblings (>80 years) of one family presented with B cell chronic lymphocytic leukemia (B-CLL). In an attempt to find common characteristics in the four patients, we performed detailed immunological marker analysis, Southern blot analysis of immunoglobulin (Ig) genes, and cytogenetic studies. In three patients clonality of the B-cells could be proven by single Ig light chain expression, but in the fourth patient no Ig light chain expression was detected and clonality of the B cells could only be demonstrated by Southern blot analysis of the Ig genes. Interestingly, in two patients, the Ig gene rearrangement patterns were compatible with the presence of two independent B cell clones, whereas in the two other siblings a monoclonal rearrangement pattern was found. All four patients showed clonal chromosome aberrations, which were different in each patient. In the two patients with biclonal Ig gene rearrangement patterns, two unrelated clones could also be demonstrated by the cytogenetic studies. These combined Ig gene and cytogenetic data indicate the presence of two different B-CLL in two of the four patients. Remarkably, the B-CLL cells of the two oldest patients expressed the CD8 antigen, which is rarely observed. Our finding of six different B-CLL in the four living siblings indicates that the members of this family are highly susceptible to the development of B-CLL. However we could not identify a common factor to explain this susceptibility further. In contrast to the literature, the occurrence of two B-CLL in one patient and the expression of CD8 were not associated with clinically aggressive disease in this family.

A patient with a variant form of hairy cell leukaemia.

A 57-year-old woman was admitted because of weakness, fatigue, abdominal discomfort, easy bruising and splenomegaly. A highly elevated leukocyte count with hairy-cell-like cells was found, the cells being positive for the monoclonal antibodies CD19, FMC7, CD11c and B-ly-7 and negative for CD24 and CD25. Blood and bone marrow were investigated not only in our own laboratory but also in several other laboratories resulting in a variety of possible diagnoses. Only after combining all data could a definitive diagnosis of variant hairy cell leukaemia be made. The patient was treated initially with a splenectomy and later on with interferon-alpha-2b, resulting in a steady decrease in the leukocyte count. After a follow-up of 2 years a nearly complete remission was obtained with a good quality of life. The differential diagnosis of this rare disorder is discussed with emphasis on the relative contribution of different diagnostic procedures.

The excretion of metabolites of testosterone and of estradiol in male patients with chronic renal failure.

Intravenous infusions of 14C-testosterone (Te) either alone or in combination with 3H-estradiol (E2) were given to five normal male subjects, twelve male patients on haemodialysis (HD) treatment and to one patient with a very restricted renal function. The elimination of radioactivity was measured in urine, HD fluid and faeces. Urinary excretion diminished with renal function. It was negligible at a creatinine clearance of less than one ml per minute. A quarter of both isotopes was eliminated by six HD treatments within three weeks. No difference was found in this respect between nephrectomized patients and those who were still in possession of their kidneys. The main excretion occurred in the stools. E2 metabolites, and to a lesser extent Te metabolites, appeared in the faeces within 24 hours, which might be explained by biliary excretion only. More 3H (E2 metabolites) than 14C (Te metabolites) was found in the faeces; more 14C than 3H found in HD fluid.

The influence of hormonal contraceptives on sex hormone binding globulin (SHBG) capacity.

PIP: The influence of oral contraceptives (OCs) on sex hormone binding globulin (SHBG) capacity was studied in women using OCs for a at least 2 months and compared with values in normal menstruating women, in pregnant women, in postmenopausal women, and in normal males. Binding capacity was measured by ammonium sulfate precipitation. The mean SHBG capacity for normal men was 28 nM/1; for normal women, 59 nM/1; and for women on OCs, 138 nM/1. Progestational OCs (d-norgestrel, lynestrenol, and medroxyprogesterone acetate) depressed the SHBG capacity of plasma by approximately 30%. .05 mg ethinyl estradiol elevated SHBG capacity (p less than .01), and when combined with either .125 mg d-norgestrel or 1 mg lynestrenol, less elevation of SHBG capacity was noted. The combination of ethinyl estradiol with megestrol acetate gave results comparable with those attained during pregnancy. It appeared that the SHBG response to OCs is different from that of transcortin-binding capacity.^ieng