Pure red cell aplasia and primary antiphospholipid syndrome: a unique association.

Pure red cell aplasia (PRCA) is a disease with important relationships to autoimmune mechanisms. Although some autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus, have been associated with PRCA, until this point no studies have described the association between PRCA and primary antiphospholipid syndrome (APS). This is the first case report of PRCA associated with primary APS in a 39-year-old man with acute heart failure secondary to an anaemic condition that was diagnosed as pure red cell aplasia. The patient was later diagnosed with retinal artery and vein thromboses and bilateral deep venous thromboses of the femoral and popliteal veins. The most common causes of PRCA and other thrombophilias were ruled out in this investigation through complementary tests. This association with APS adds a new possibility to the study of PRCA pathophysiology.

[Pure red cell aplasia after allogeneic transplantation of ABO incompatible hematopoietic stem cells]. / Aplasia pura de serie roja post-trasplante alogeneico de células progenitoras hematopoyeticas ABO incompatible.

ABO incompatibility in allogeneic bone marrow transplantation may be associated with incomplete or delayed erythroid engraftment, being pure red cell aplasia (PRCA) the most severe complication in this setting. Attempts for the treatment of PRCA have been made with erythropoietin or with plasmapheresis with relative success, and some authors have reported the reversibility of PRCA with antilymphocyte globulin (ALG or ATG), based on the assumption that PRCA might be immunologically mediated. We report herewith a patient with acute leukemia who developed post--BMT pure red cell aplasia. His sibling donor (sister) was HLA identical and ABO incompatible, having low agglutinin titers against donor's blood group. PRCA did not improve after treatment with erythropoietin or a boost of hematopoietic progenitor cells obtained from donor's peripheral blood but the problem was resolved completely after treatment with ALG.

Aplasia pura de serie roja post-trasplante alogeneico de células progenitoras hematopoyeticas ABO incompatible / Pure red cell aplasia after allogeneic transplantation of ABO incompatible hematopoietic stem cells

El trasplante alogeneico de células progenitoras hematopoyéticas (TCPH) con incompatibilidad ABOentre el donante y el receptor puede en ocasiones asociarse a trastornos en la progenie eritroide de-sarrollada a partir de la médula ósea trasplantada, caracterizado por un funcionamiento tardío, inadecuado e in-completo de la misma. En este contexto, la aplasia pura de serie roja es la complicación más severa. Se han inten-tado tratamientos para la aplasia pura de serie roja post-TCPH con eritropoyetina o plasmaféresis, con relativo éxito. Algunos autores han informado también la utilización de globulina antilinfocitaria, asumiendo que dicha aplasia se-lectiva de la serie roja en la médula ósea trasplantada es mediada por un mecanismo inmune. En este trabajo se describe un paciente portador de una leucemia aguda en quien se realizó un TCPH alogeneico (ABO incompatible con su donante). Teniendo niveles bajos de aglutininas contra el grupo sanguíneo de la donante, desarrolló una aplasia pura de serie roja post - TCPH. La misma no mejoró con tratamiento con eritropoyetina o con un refuerzo de progenitores hematopoyéticos de sangre periférica de la misma donante ( boost), resolviéndose totalmente lue-go de un tratamiento exitoso con globulina antilinfocitaria de origen equino (AU)

Comparison of the erythropoietin response in children with aplastic anemia, transient erythroblastopenia, and iron deficiency.

To assess the effects of decreased erythrocyte production on the levels of serum erythropoietin in children, we measured simultaneous hemoglobin concentrations and erythropoietin in 18 children with iron deficiency anemia, 17 children with transient erythroblastopenia of childhood (TEC), and 7 children with aplastic anemia. In all but two patients (one with TEC; one with aplastic anemia), erythropoietin was measured at diagnosis, before institution of specific therapy for the anemia. There was a statistically significant inverse linear correlation between log10 erythropoietin and hemoglobin values for all patient groups (r = 0.904 to 0.912; p less than 0.005). A comparison of the slopes of the regressions for each patient group by analysis of covariance revealed a significantly steeper slope for the iron deficiency group (-0.553) versus the TEC (-0.287) and aplastic anemia (-0.256) groups (p = 0.0001). The difference in erythropoietin levels appeared greatest in patients whose presenting hemoglobin level was greater than 5 gm/dl. Decline in serum erythropoietin levels was more precipitous in the less severely anemic patients with iron deficiency anemia than in the patients with TEC or aplastic anemia. These data reveal quantitative and qualitative differences in the relationship between serum erythropoietin and hemoglobin levels when children with severe iron deficiency anemia versus those with TEC or aplastic anemia are considered, even though all three conditions are characterized by hypoproliferation of erythrocytes.

Pure red cell aplasia and hepatitis in a child receiving isoniazid therapy.

A 7-year-old boy had pure red cell aplasia and clinically significant hepatitis during isoniazid therapy. The former complication had been reported only in adults, and the latter is rare in children.

G gamma-levels of the HbF of patients with bone marrow failure syndromes.

1. The gamma chain composition of 17 patients with varying degrees of bone marrow failure was determined by high performance liquid chromatography. All 10 patients with anemia had high G gamma levels (48.1-68.5%), while 5/7 patients in remission had G gamma in the range 30.0-38.8%. 2. There was also an association of higher G gamma values with higher HbF levels. The average value of G gamma in the HbF of patients with active aplastic or hypoplastic anemia differed from those obtained both for normal adult and for newborn HbF. 3. Thus, in spite of activation of both the G gamma and A gamma genes, the former are more effectively expressed as HbF increases.