Challenges in providing compatible blood with Rh genotype-matching in Brazilian patients with sickle cell disease.

OBJECTIVES: The aim of this study was to verify the possibility of performing prophylactic Rh genotype-matching in Brazilian patients with sickle cell disease (SCD) and identify the genotypes that are lacking or insufficient in our donor cohort. BACKGROUND: Rh alloimmunisation is still a challenge in transfused patients with SCD. Rh genotype-matching may mitigate Rh alloimmunisation. METHODS/MATERIALS: We examined the transfusion requests for antigen-matched donor units in SCD patients with Rh variants and compared the Rh altered alleles in the patients to the Rh allele frequency in a selected donor population. For each patient and donor, RBC antigen genotyping was performed using HEA, RHD and RHCE BeadChip arrays. Sequencing was used to clarify inconclusive results. Twenty-one patients and 956 Brazilian blood donors were genotyped. RESULTS: According to our matching strategies, 47·6% of patients filled most of their unit requests, but 52·4% of patients had insufficient donors to fill their annual transfusion needs. We found different combinations of RHCE variant alleles in patients and donors, but the most frequent genotypes that are lacking or insufficient in our donor cohort are those associated with the lack of hrB and hrS high prevalence antigens and those co-inherited with altered RHD alleles. CONCLUSION: Our study shows that the provision of compatible blood with Rh genotype-matching in Brazilian patients with SCD can be feasible but challenging and, efficient strategies of recruitment of African-Brazilian donors must be developed.

Third-trimester erythrocytapheresis in pregnant patients with sickle cell disease.

OBJECTIVE: To evaluate the effects of prophylactic transfusion by means of erythrocytapheresis at the beginning of the third trimester of pregnancy in women with sickle cell disease (SCD). METHODS: A cohort of 14 pregnant women with SCD who received prophylactic erythrocytapheresis transfusions at the beginning of the third trimester was retrospectively compared with a cohort of 17 pregnant women who received simple prophylactic transfusions for no indication other than SCD severity. RESULTS: Prophylactic erythrocytapheresis transfusions were associated with a lower risk of intrauterine growth restriction (OR, 0.11; 95% confidence interval, 0.01-1.00) and oligohydramnios (OR, 0.65; 95% confidence interval, 0.45-0.92) in pregnant women with SCD. CONCLUSION: These results suggest that erythrocytapheresis transfusions are beneficial in women with SCD who are in the third trimester of pregnancy. Given the decrease in transfusion risks, this therapy deserves further evaluation in future trials.

[Surgical treatment of fungal bolus in acute lymphoid leukemia]. / Tratamento cirúrgico de "bola fúngica" em leucemia linfóide aguda (LLA).

Case report of a 24 year old female patient with ALL that developed pulmonary invasive aspergillosis during aplastic phase of induction chemotherapy. She was treated with antibiotics and amphotericin B. After recovering from neutropenia, she developed a mycetoma in the inferior lobe of the right lung, which required lobectomy. Nine months after surgery the patient is well, in complete remission of ALL and with no evidence of infection. One month after lobectomy, chemotherapy had been reintroduced. Attention should be called to this form of therapy of Aspergillosis, as a successful way to eradicate this fungal infection that responds poorly to antifungal drugs currently used.

Decreased GATA3 mRNA expression in human T-cell lymphotropic virus type 1 (HTLV-1) infection.

GATA3 is a specific T-cell transcription factor involved in the expression of T-cell receptor (TCR). In order to characterize the relationship between HTLV-1 infection, which has been reported to be associated with down-regulation of genes belonging to the TCR/CD3 complex, and the transcription factor GATA3, we evaluated, by semi-quantitative RT-PCR, the expression of GATA3 gene in HTLV-1 carriers and individuals with related diseases. The study included 4 asymptomatic carriers, 2 patients with adult T-cell leukaemia/lymphoma (ATLL), 1 patient with HTLV-1 associated myelopathy (HAM)/tropical spastic paraparesis (TSP) and 7 healthy blood donors. A considerable decrease in the expression of the GATA3 mRNA was observed in all subjects infected by HTLV-1 and no expression of GATA3 mRNA was observed in 1 subject with ATLL and in 1 with HAM/TSP.

Severe immune haemolysis in a group A recipient of a group O red blood cell unit.

Haemolysis caused by passive ABO antibodies is a rare transfusional complication. We report a case of severe haemolytic reaction in a 38-year-old man (blood group A) with lymphoma who had received one red blood cell (RBC) unit group O. After transfusion of 270 mL, the patient experienced fever, dyspnoea, chills and back pain. On the following morning he was icteric and pale. Haptoglobin was inferior to 5.8 mgdL(-1), haemoglobin was not increased and lactate dehydrogenase was elevated. Haemolysis was evident on observation of the patient's post-transfusion samples. The recipient's red cells developed a positive direct antiglobulin test and Lui elution showed anti-A coated the cells. Fresh donor serum had an anti-A titre of 1024, which was not reduced by treating the serum with dithiothreitol. Donor isoagglutinin screening has been determined by microplate automated analyser and showed titre higher than 100. Physicians should be aware of the risk of haemolysis associated with ABO-passive antibodies. There is generally no agreement justifying the isoagglutinin investigation prior to transfusion. However, automated quantitative isoagglutinin determination could be part of the modern donor testing process, mainly in blood banks where identical ABO group units (platelets or phenotyped RBCs) are not available owing to limited supply.

Tratamento cirúrgico de "bola fúngica" em leucemia linfóide aguda (LLA) / Surgical treatment of fungal bolus in acute lymphoid leukemia

Case report of a 24 year old female patient with ALL that developed pulmonary invasive aspergillosis during aplastic phase of induction chemotherapy. She was treated with antibiotics and amphotericin B. After recovering from neutropenia, she developed a mycetoma in the inferior lobe of the right lung, which required lobectomy. Nine months after surgery the patient is well, in complete remission of ALL and with no evidence of infection. One month after lobectomy, chemotherapy had been reintroduced. Attention should be called to this form of therapy of Aspergillosis, as a successful way to eradicate this fungal infection that responds poorly to antifungal drugs currently used.