Gamma delta (γδ) T-cell antigen receptor (TCR) expression and its related T-cell differentiation are not commonly reported in T-cell acute lymphoblastic leukemia/lymphoma (T-ALL). Here we report two pediatric T-ALL cases and present their clinical features, histology, immunophenotypes, cytogenetics, and molecular diagnostic findings. The first patient is a two-year-old girl with leukocytosis, circulating lymphoblasts, and a cryptic insertion of a short-arm segment at 10p12 into the long-arm segment of 11q23 resulting in an MLL and AF10 fusion transcript, which may be the first reported in γδ T-ALL. She responded to the chemotherapy protocol poorly and had persistent diseases. Following an allogeneic bone marrow transplant, she went into remission. The second patient is an eleven-year-old boy with a normal white cell count, circulating blasts, and a normal karyotype, but without any immature cellular markers by flow cytometric analysis. He responded to the chemotherapy well and achieved a complete remission. These cases demonstrate the diverse phenotypic, cytogenetic, and molecular aspects of γδ T-ALL. Early T-precursor- (ETP-) ALL and their differential diagnosis from other mature γδ T-cell leukemia/lymphomas are also discussed.
Background:
Chronic red blood cell transfusion has been proven to be effective in prevention of strokes, silent cerebral infarcts, acute chest syndrome, recurrent priapism and in pregnancy. The use of regular transfusions to mitigate other morbidities of sickle cell disease (SCD) is evolving. In the silent infarct transfusion (SIT) trial in children, chronic transfusion lead to a significant improvement in quality of life. Some of the common reasons patient with SCD do not get chronic transfusion is fear of alloimmunization, iron over load and risk of viral infections. We did a retrospective analysis of adult patients with SCD who need chronic blood transfusion to determine the incidence of alloimmunization. At our institute all pediatric sickle cell patients needing chronic transfusion are placed on protocol, receive C, E, and K matched blood, and remain on the protocol until they become adults.
Methods:
We electronically collected data from 180 adult SCD patients who need chronic transfusions and analyzed the data for the number of transfusions received, incidence of allo- immunization and most common antibodies identified.
Results:
A total of 3967 red blood cell transfusions were administered on 180 adult sickle cell disease patients. Twenty five patients developed antibodies (13.8 %). Fifteen out of the 25 had multiple antibodies (60%). The alloantibodies identified were : anti- K(11), anti- E(12), anti- Fya(5), anti-C (4), anti-V (4), anti- S (3), anti-D (2), anti- Jkb (1), anti-Jsa(1) , and anti- Lutherana (3). Two patients had cold and 5 patients had warm autoantibodies.
Conclusion:
The policy to place patients with SCD needing chronic transfusion on protocol to receive C, E, and K matched red blood cells has decreased the alloimmunization rate to 13.8 %. We conclude that, fear of alloimmunization should not preclude physicians from using chronic red cell transfusions to prevent complications in sickle cell disease.
Disclosures
No relevant conflicts of interest to declare.
A bone marrow clot section comprised of sinusoidal blood is inadequate for morphological interpretation and is a waste of resources. Hence, we recommend the cell block technique for procurement; this method ensures maximum capture of material needed to establish a diagnosis.
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