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Benign familial (hereditary) leukopenia and neutropenia (BFLN) have been reported in some ethnic groups, including black Americans and Yemenite Jews. The bone-marrow response of 34 Yemenite Jews (with and without neutropenia) to an intravenous injection of 200 mg of hydrocortisone sodium succinate was studied and compared with the response of 18 healthy control subjects. The mean +/- SEM of the increments in polymorphonuclear cells (PMNs) following injection of hydrocortisone in Yemenite Jews (2,413 +/- 245/mm3 in neutropenic subjects and 2,187 +/- 343/mm3 in nonneutropenic subjects) were significantly lower than in the control subjects (4,431 +/- 467/mm3), without significant differences among the subgroups of the Yemenite Jews. The decreases in monocytes, lymphocytes, and eosinophils were similar in all groups. No correlation was found between baseline PMN levels and the increments following hydrocortisone administration. These results suggest a lowered bone-marrow response to hydrocortisone in subjects with BFLN, indicating some defect in PMNs release from the bone-marrow storage pool to the peripheral blood. It seems that this defect characterizes all members of the ethnic group, whether they have "overt" neutropenia or not.

Benign neutropenia is often found among healthy Yemenite Jews. An assessment was made of the magnitude of labor-induced leukocytosis in 44 Yemenite Jewish women, by comparing their hematological values during labor with those of 27 non-Yemenite women. A statistically significant difference was found in the absolute counts of the white blood cells (WBC) during delivery between the two groups, the values being lower among Yemenite Jews [10,291 +/- 422] vs. [11,759 +/- 630] X 10(9)/1) and similar findings were detected among their infants. The low WBC counts in the Yemenite group at parturition were also associated with significantly lower plasma cortisol levels (Yemenite group: 33.7 +/- 3.5 micrograms/dl; controls: 49.3 X 2.9 micrograms/dl). A correlation was found between serum cortisol levels and the magnitude of leukocytosis in both groups of women and their infants. These results suggest that a low basal corticosteroid output may contribute to the low white blood cell counts detected in some ethnic groups like the Yemenite Jews.

The HLA antigenic system was studied in Yemenite Jews with genetic neutropenia. No deviation in antigen frequency was observed when compared with matched controls from the same ethnic group. HLA antigen frequency and common haplotypes of the Yemenite Jews were found entirely different from those of the African blacks, known to have the same anomaly.

The components of the peripheral white blood cells (WBC) were determined before and after 10 min of submaximal ergometric work in two groups of subjects: 34 healthy Yemenite Jews with benign familial leukopenia (BFL) and 34 healthy Yemenite Jews without BFL. The mean +/- SEM of the increase in the peripheral WBC following the effort in the control group was 2,606 +/- 1,272 cells/mm3, due to the relative rise in the neutrophils, eosinophils, monocytes and lymphocytes. The identical ergometric work, performed by BFL subjects, brought about significantly lower increase (700 +/- 865 cells/mm3) in the peripheral WBC, the majority of which were lymphocytes. It seems that subjects with this form of BFL lack granulocytes in the marginal pool.

Benign familial leukopenia (BFL) is a hereditary phenomenon, encountered in several ethnic groups. Subjects bearing BFL are believed to be affected by bacterial infection in no greater incidence than normal subjects. In our study we investigated a group of subjects with BFL during an acute bacterial infection in comparison to subjects without BFL with the same infection. We found that the subjects with BFL had no absolute leukocytosis during the infection. Nevertheless, they reacted similarly to the other subjects in regard to their temperature and heart rate; however, they were hospitalized for fewer days than subjects without BFL. We conclude that BFL is a benign phenomenon, requiring neither specific treatment as such, nor more aggressive therapy during infection. The benign course of an acute bacterial infection in BFL indicates that perhaps the number of WBC's that are normally recruited during an infection in normal subjects highly exceeds that which is necessary.