Neutropenia in 6 ethnic groups from the Caribbean and the U.S.

BACKGROUND: Low white blood cell counts (WBC) or absolute neutrophil counts (ANC) may delay or prevent the completion of appropriate chemotherapy, especially among women receiving adjuvant therapy for breast and colon cancer, and affect cancer survival. Because race/ethnicity is also associated with survival, the authors compared WBC and ANC in healthy American-born women of African descent and European descent, and women from Barbados/Trinidad-Tobago, the Dominican Republic, Haiti, and Jamaica. METHODS: Blood samples from 261 healthy women ages 20 to 70 years were tested for WBC with differential, cytokine and growth factor levels, and ancestry informative and neutrophil elastase polymorphisms. The authors analyzed the association between neutropenia and serum WBC growth factor levels, cytokine levels, and neutrophil elastase c199a polymorphism. RESULTS: The median WBC and ANC differed among the 6 groups (P < .01 for WBC and P < .0001 for ANC). Dominicans were found to have higher median WBC and ANC than all other groups (P < .03). Neutropenia (ANC < 1500 cu/mm) was observed among 2.7% to 12.5% of the groups of predominantly African descent; no other groups were found to have neutropenia (P < .05). Granulocyte-colony-stimulating factor was found to be lower in white women, but tumor necrosis factor-alpha and C-reactive protein were not found to be correlated with ethnicity. Women of African origin were more likely to have polymorphisms of African ancestry (P < .001) and c199a alleles (P < .0001), which were also associated with low ANC levels. CONCLUSIONS: In the current study, the authors observed a strong association between neutropenia and African descent among asymptomatic women from the U.S. and the Caribbean. Among women of African descent who develop a malignancy, this association may contribute to racial disparities in treatment and outcomes.

Stroke in a cohort of patients with homozygous sickle cell disease

Strokes occurred in 17 of 310 children with homozygous sickle cell disease who were followed from birth, representing an incidence of 7.8 percent by the age of 14 years. Two children had subarachnoid hemorrhage, one having resolution of symptoms after aneurysm surgery and another dying of a presumed second hemorrhage 14 days later. The remaining 15 strokes were presumed to be cerebral infarction, although autopsy, angiographic, or computed tomographic evidence was available in only 8 children. There were 6 deaths, 2 in the acute events and 4 after recurrence, which occurred in 6 (46 percent) of 13 patients who survived the initial episode. There were 10 recurrent episodes at a median interval of 9 months after the initial event. Steady-state hematologic data revealed significantly higher leukocyte counts than in control subjects without strokes at age 1 year and in the last study preceding the stroke. The initial stroke coincided with an acutely lowered hemoglobin value in 5 patients (3 aplastic crises, 1 acute splenic sequestration, 1 probable pulmonary sequestration) and with painful crises in another 7 patients. We conclude that a high leukocyte count and an acute decrease of hemoglobin are risk factors for stroke in patients with homozygous sickle cell disease.(AU)

Haematological change in sickle cell-haemoglobin C disease and in sickle cell-beta thalassaemia: a cohort study from birth

The haematological changes in early years following neonatal diagnosis have been observed in representative groups of children with sickle cell-haemoglobin C (SC) disease, sickle cell-á+ thalassaemia, and in sickle cell-á Thalassaemia. Most haematological indices in SC disease were intermediate between previously published values in SS disease and in AA controls, generally being closer to values in normal children, Eceptions were microcytosis which may be genetically determined and a striking elevation of mean cell haemoglobin cocentration from age 2 months to 4 years. The combination of a raised MCHC and a lowered MCV is unusual and may be characteristic of SC disease. Features in sickle cell-á thalassaemaia generally differed accordingly to the type of á thalassaemia gene. Sickle cell-B degree thalassaemia had lower levels of haemoglobin, MCHC, red cell count, MCV, and higher reticulocytes, most differences being significant before 1 year. No differences between SB degree thalassaemia and Sá+ thalassaemia were apparent in HbF levels (which resembled those in SS disease) or in HbA2 levels (which exceeded those in SS disease by 1 year of age).(AU)

The haematology of steady state homozygous sickle cell disease: frequency distibutions, variation with age and sex, longitudinal observations

The steady state haematological characteristics observed in 1071 patients with homozygous sickle cell (SS) disease aged 5-66 years are presented (Summary)

Coagulation changes during the steady state in homozygous sickle-cell disease in Jamaica

Coagulation studies were carried out in 117 Jamaicans with homozygous sickle-cell disease in the steady state, and 40 local controls. The patients had significantly higher factor-VIII levels, higher platelet counts, lower factor-V and plasminogen levels, shorter thrombin times and higher serum fibrinogen degradation products(FDP) than the control group. The low factor-V and plasminogen levels, and high FDP levels, might be explained by activation of the coagulation system and continuous clot lysis even in the absence of painful crisis. The high factor-VIII levels and short thrombin times found in these patients could not be explained.(Summary)

Delayed skeletal maturation in sickle cell anemia in Jamaica

The maturation of the left distal radial epiphysis was studied in 86 adolescent patients with homozygous sickle cell disease in Jamaica. Development was retarded in 81 patients and in the remaining five, the epiphysis had fused. Delay in epiphyseal fusion related to the level of fetal haemoglobin. Delay in epiphyseal fusion may be an important determinant of the characteristic habitus of adults with sickle cell anemia.(Summary)

The iron status of pregnant women with hemoglobinopathies

In a study of the iron status of 15 pregnant women with hemoglobinpathies, eight had no sustainable iron in the bone marrow, despite the absence of acute or chronic blood loss, intercurrent infection, or infestation with parasites; in three, iron deficiency was present before the the sixteenth week of pregnancy. This study shows that in our population the prevention of iron deficiency is important in pregnant women with hemoglobinpathies (AU)

Splenic histiocytosis associated with thrombocytopenia

Two young Jamaican women of Chinese descent with idiopathic thrombocytopenic purpura were found to have lipid-laden histiocytes in their spleens; their condition has improved following splenectomy. The electron microscopic appearances of the spleens suggest that platelet destruction contributed to the accumulation of lipid (AU)

Symptoms and clinical and laboratory findings in 123 cases of Schistosomiasis mansoni in St. Lucia

The presenting symptomatology and clinical and laboratory findings in 123 patients, referred to a clinic for the treatment of schistosomiasis mansoni, are reported and discussed. Thirty-three patients (26.8 per cent) had no complaint when seen initially. The complaints of the others, in order of frequency, were abdominal pain, weakness, diarrhoea (with or without blood in faeces), giddiness, anorexia, dyspnoea and fever. The mean faecal egg load of patients with bloody diarrhoea was not significantly higher than that of patients with diarrhoea alone. Eighteen patients had clinical enlargement of the liver (14 or 11.4 percent) and of the spleen (4 or 3.2 percent) which could be ascribed to schistosomiasis. Hepatosplenomegaly in three other patients as ascribed to other conditions. The mean faecal egg concentration was significantly higher in patients with hepatosplenomegaly than in those with hepatomegaly alone. Ranges and mean values of haemoglobin and eosinophilia are given by age group. Alteration in plasma proteins was found in patients who had spleen and/or liver enlargement, the latter group having a reversed albumen/globulin ratio which, in the two instances in which electroporesis could be carried out, was found to be the result of a tremendous increase in the gamma globulin fraction.(Summary)