Hematological features in children with systemic lupus erythematosus: are they more common than appreciated?

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Varicella Zoster-Associated Severe Aplastic Anemia in a Child and Its Successful Treatment with Peripheral Blood Stem Cell Transplantation from HLA-5/6-Identical Donor

Background: Varicella zoster virus is very rarely associated with aplastic anemia. Bone marrow transplantation from an HLA-identical sibling is the treatment of choice. Case report: A seven-year-old boy presented with aplastic anemia (AA) following chicken pox infection. No clinical improvement was observed with pharmaceutical therapy and peripheral blood stem cell transplantation (PBSCT) was performed from his HLA 5/6 identical mother. Since the transplantation, the patient has had a durable, trilineage hematological response for 12 months. Conclusions: The present case with varicella zoster-associated aplastic anemia was non-responsive to conventional therapies (ATG protocol, ALG protocol, oxymethalone, and cyclosporine A) and successfully treated with bone marrow transplantation from his 5/6 identical mother.Wo

Hyperacute Graft-vs.-Host Disease After Related HLA-Identical Umbilical Cord Blood Transplantation

haGVHD has been described following bone marrow and peripheral blood stem cell transplantation and in a single case who received unrelated HLA mismatched CB. An unusual case of haGVHD following HLA 6/6-matched sibling CBT in a child with AML is presented. The development of haGVHD in a fully matched CBT and without precipitating factors may suggest the role of still undefined and perhaps individual contributory factors

Molecular Diagnosis Of Shwachman-Diamond Syndrome Presenting With Pancytopenia At An Early Age: The First Report From Turkey

A three-month-old boy presented with growth failure, skeletal abnormalities, otitis media and pancytopenia. Exocrine pancreatic insufficiency was confirmed by low levels of fecal elastase. He was diagnosed as Shwachman-Diamond syndrome by clinical and laboratory findings. The diagnosis was confirmed by sequence analysis for SBDS gene on chromosome seven revealing compound heterozygous mutation, which are c.258+2T-C and c.183-184TA-CT. Matched unrelated donor screening for hematopoietic stem cell transplantation was initiated. Unfortunately, he died of respiratory difficulty at 5 months of age. Our case is the youngest patient whose presumptive Shwachman-Diamond syndrome diagnosis was confirmed by molecular analysis.Wo

The evaluation of acquired aplastic anemia in children and unexpected frequency of varicella-zoster virus association: a single-center study.

Wo

The Effect of Glutamine Supplementation on Hematopoietic Stem Cell Transplant Outcome in Children: A Case-Control Study

HSCT associated morbidity and mortality is usually attributed to high-dose chemotherapy/radiotherapy regimens used for conditioning. Glutamine (Gln), a conditionally essential amino acid during severe catabolic states, has been shown to have favorable effects in patients with malignancies and in those undergoing HSCT. However, controversy exists regarding its routine use. Studies in children investigating gln supplementation are very limited. In the present study, including 21 gln-supplemented and 20 control pediatric patients, gln supplementation was shown to reduce the duration of fever and decrease the incidence of SOS during the HSCT course. In addition, a decrease in drug-related toxicity and a trend toward reduced incidence of severe mucositis were observed.Wo

Serum Erythropoietin Levels In Pediatric Hematologic Disorders And Impact Of Recombinant Human Erythropoietin Use

Objective: In anemic patients, the correlation between serum erythropoietin (sEpo) level and the severity of anemia has been reported previously. However. in different anemia groups, different sEpo levels are measured in patients with similar hemoglobin levels and the etiology of this situation could not be explained. Methods: We evaluated hemoglobin and sEpo levels in 31 iron deficiency anemia, 26 Fanconi anemia (FA), 21 thallasemia intermedia (TI), 15 acute lymphoblastic leukemia (ALL) patients at presentation and 12 healthy controls. Results: In all disease groups, an inverse linear correlation was shown between hemoglobin and logarhytmic sEpo level. The covariance analyses according to corrected hemoglobin levels exhibited the highest sEpo level in FA, followed by ALL, TI and iron deficiency anemia, sequentialy. Conclusion: There was no statisticaly significant difference of sEpo levels in FA patients in terms of androgen treatment and this finding supports that androgen affects erythropoisis directly, and has no effect on erythropoietin. The results indicate that there is no erythropoietin deficiency in the anemia of these patients and the admnistration of exogenous erythropoietin offers no clinical benefit. (Turk J Hematol 2009; 26: 72-6)Wo

A Prompt Graft-Versus-Thalassemia Effect upon Withdrawal of CyclosporineA in A Child Who Received Allogeneic Peripheral Blood Stem Cell Transplantation

Stable mixed chimerism is a common event in children with thalassemia major who have undergone bone marrow transplantation (BMT). However, persistence of unstable mixed chimerism in these children is associated with a greater risk of rejection and/or relapse of the disease. Andreani et al.1 has reported that rejection and/or disease recurrence occurs in approximately one third of patients with early mixed chimerism in thalassemia major.Wo

Atypical Skin Graft-vs.-Host Disease Following Bone Marrow Transplantation in an Infant

Herein, we describe an unusual presentation of acute graft versus host disease (GVHD) mimicking contact dermatitis in an infant who underwent 5/6 HLA-matched bone marrow transplantation (BMT) from his mother for malignant infantile osteopetrosis. The initial rash on day +32 simulated diaper rash, which progressed to a belt-shaped rash and then developed hyperkeratotic nodules on the hands. The acute GVHD was atypical and the course was progressive and fatal, with liver and gut involvement. This presentation of atypical initial skin involvement of acute GVHD may be useful for practicing clinicians in the BMT field who need to be aware of the early unusual signs of acute GVHD so that they can initiate prompt treatment