Decreased stainable marrow iron in chronic granulocytic leukemia.

Stainable iron was absent or decreased in 36 of 45 bone marrow biopsy specimens (80 percent) among 33 patients with chronic-stage chronic granulocytic leukemia. Decreased iron did not correlate with sex, treatment status, duration of disease, marrow cellularity, or hemoglobin level. In contrast, marrow iron was absent or decreased in 34 percent of biopsy specimens at diagnosis of acute nonlymphocytic leukemia (p less than 0.0001) and 31 percent of biopsy specimens from patients with Hodgkin's disease (p less than 0.0001). The serum ferritin level was determined in eight patients with chronic granulocytic leukemia and absent marrow iron, and it was normal in all. Fifteen of 17 patients, followed with chronic-stage disease for one to four years after the finding of absent marrow iron, demonstrated increases in their hemoglobin levels during antileukemic therapy or maintained normal values. Thus, absent or decreased stainable marrow iron is a common finding in chronic granulocytic leukemia and usually does not indicate iron deficiency.

Antibody responses to protein, polysaccharide, and phi X174 antigens in the hyperimmunoglobulinemia E (hyper-IgE) syndrome.

To investigate whether an underlying defect in antibody (Ab)-forming capacity could contribute to the infection susceptibility of patients with hyper-IgE syndrome, we evaluated 11 such patients for their responses to bacteriophage phi X174 (phi X174), diphtheria and tetanus toxoids, and pneumococcal (Pneumovax) and Hemophilus influenzae vaccines. Three of nine patients immunized with phi X174 had normal primary and secondary Ab responses, five had accelerated declines in their titers after initially normal primary Ab responses and lower than normal secondary Ab responses, and two of the latter patients failed to switch normally from IgM to IgG Ab production. Only one of 10 patients tested had normal Ab responses to diphtheria toxoid, and postimmunization antitetanus titers were abnormally low in five of the 10 patients tested. Serum Abs to H. influenzae polyribose phosphate were protective in seven of the eight immunized patients. Five of the nine patients administered Pneumovax had poor Ab responses to at least one of the pneumococcal serotypes 7, 9, or 14. Abnormal antipolysaccharide responses did not correlate with IgG2 deficiency. All patients responded with protective Ab levels to type 3. Thus, patients with hyper-IgE syndrome are heterogeneous with respect to their Ab-forming capacities. Ab deficiency may contribute to infection susceptibility in some of these patients.

HLA-B27-associated arthropathy in childhood: long-term clinical and diagnostic outcome.

We conducted HLA-B27 tissue typing assessments on 430 consecutive children whose main symptom at presentation to our clinic was arthritis/arthralgia. Eighty-five of them (20%) had the B27 antigen. Thirty-six of these children were reexamined after a mean followup period of 8.9 years. Although most had definable rheumatic diseases, only 2 met the New York criteria for ankylosing spondylitis (AS). Children with HLA-B27 and arthritis/arthralgia, although at increased risk of developing AS, have diverse diagnostic and clinical outcomes. The AS criteria used to diagnose the disease in adults may not be appropriate for use in children.