Shyam Rathi

Association between lower hair zinc levels and Neural Tube Defects

Though folic acid supplementation has reduced the incidence of Neural Tube Defects (NTD), NTD still constitutes one of the important congenital malformations having wide medical, social and ethical implications. Zinc deficiency has been reported to produce NTD in animals. This study was designed to evaluate zinc status of the newborn babies with NTD and their mothers. Eighty newborn babies with NTD and their mothers served as cases. Eighty apparently normal newborn babies and their mothers served as controls. Serum and scalp hair zinc levels were analyzed by atomic absorption spectrophotometry. The mean (± SD) serum and hair levels in normal mothers were 74.1 ± 4.1 μg/dl and 142.3 ± 8.0 μg/g respectively. The mean (± SD) serum and hair levels of the mothers who delivered NTD babies were 75.7 ± 5.6 μg/dl and 129.9 ± 5.3 μg/g respectively. The mean (± SD) serum and hair levels in normal newborn babies were 77.8 ± 5.3 μg/dl and 188.8 ± 6.2 μg/g respectively. The mean (± SD) serum and hair levels in NTD babies were 80.1 ± 12.9 μg/dl and 174.2 ± 10.7 μg/g respectively. The hair zinc levels of the affected babies and their mothers were significantly lower (P< 0.001) than the controls. This study has found association between NTD and decreased hair zinc levels and large population based studies are recommended to confirm the association between zinc and NTD and to investigate whether zinc supplementation would reduce the overall incidence of NTD.

Acute renal failure in a patient with acute lymphoblastic leukemia: A rare cause

A young adult was diagnosed to have acute lymphoblastic leukemia, T-cell immunophenotype and was initiated on chemotherapy. He presented with acute renal failure two days after the completion of his induction regimen. A renal biopsy showed features of necrotizing crescentic glomerulonephritis (GN). Serology for c-anti-neutrophil cytoplasmic antibody (ANCA) was positive and a final diagnosis of ANCA-associated necrotizing crescentic GN was made. Aggressive immunosuppression could not be used due to the presence of nosocomial pneumonia and the patient expired 26 days after the renal biopsy diagnosis. We report for the first time the association of acute lymphoblastic leukemia with crescentic GN and, hence, expand the list of malignancy-related ANCA-positive GN.

Acute renal failure following antithymocyte globulin therapy for aplastic anaemia—report of two cases and review of literature

Dear Editor, Immunosuppressive therapy with antithymocyte globulin (ATG) with ciclosporine is the standard of care in patients with aplastic anaemia who are not eligible for bone marrow transplantation. ATG is an animal protein and its predominant toxicities include allergic reactions and serum sickness. Acute renal failure attributable to ATG has rarely been described. We report two patients with aplastic anaemia who developed acute renal failure during ATG therapy. Both patients developed oliguric acute renal failure with temporal relation to ATG administration and absence of other etiological factors. One of the patients had complete recovery of renal function with supportive treatment while the other died of sepsis and multi-organ dysfunction.

An unusual presentation of eosinophilic variant of chronic myeloid leukemia (eoCML)

Dear Editor, Eosinophilia, a common sign of chronic myeloid leukemia (CML) and often used as one of the criteria for accelerated phase, but there is paucity of literature with eosinophilia as the only presentation of CML. Whether these patients clinically resemble with other hypereosinophilic syndromes (HESs) is not known. High eosinophil count can cause heart, lung, and central nervous system damage in both the eosiniphilic variant of CML (eoCML) and HES. Superficial venous thrombophlebitis (SVT) is usually a benign disorder but can be the first manifestation of hypercoagulable states. Secondary causes of SVT must be sought, particularly when SVT is recurrent or multifocal or has spontaneous onset. Among the various risk factors of SVT, vasculitis, malignant blood disorders, and solid tumors are the commonest. Association of eosinophilia with SVT is a rare situation that can reveal neoplasia, but it has never been described in eoCML, which prompted present communication. A 55-year-old man presented with a history of recurrent subcutaneous swelling of the right upper limb and anterior chest wall for 3 months. Each time, the swelling used to last from a few days to few weeks. There was history of itching and sometimes pain over the swollen area. There was no response to therapy with antihistaminics or analgesics. On examination, there was ill-defined erythematous swelling, with local rise of temperature over the right forearm extending to anterior chest wall. There were palpable, hardened, and painful erythematous veins over the right forearm. Vital signs were stable. Chest and neck examination was remarkable. Therewas no lymphadenopathy or hepatosplenomegaly. Hemogram showed white blood cell count of 12,300/mm with 10% neutrophils, 5% lymphocytes, and 85% eosinophils. The absolute eosinophil count was 10,455/mm. His peripheral blood picture revealed only eosinophilic leukocytosis with normocytic normochromic red blood cells and adequate platelets. Ultrasound color Doppler showed multiple foci of superficial vein thrombosis in the swollen areas without any evidence of deep-vein thrombosis. Magnetic resonance imaging of the right upper limb showed extensive subcutaneous and intramuscular edema with superficial thrombophlebitis. He was further investigated for causes of eosinophilia and recurrent superficial thrombophlebitis. Stool culture for ova and parasites were negative. Chest X-ray and contrast-enhanced computed tomography of the chest and abdomen were normal. Serum carcinoembryonic antigen and prostate-specific antigen levels were also normal. His lipid profile, antinuclear antibodies, anticardiolipin antibodies, lupus anticoagulant, protein S, protein C, and homocysteine levels were also found to be within normal limits. Echocardiography revealed early features of endomyocardial fibrosis, and pulmonary function test was normal. Bone marrow aspirate and biopsy showed a hypercellular marrow with myeloid hyperplasia and increased number of eosinophilic precursors. His peripheral blood and bone marrow aspirate was sent for reverse transcriptase polymerase chain reaction for FIP1L1-PDGFRα and bcr–abl, for detection of any clonal abnormality. The hybrid transcript for FIP1L1-PDGFRα was absent. At presentation, the patient’s BCR–ABL/ABL ratio was 10%. Fluorescent in situ hybridization was performed for BCR/ABL gene fusion, which showed that 60% of cells were positive for this gene rearrangement. On the basis of clonal evidence, a diagnosis of eoCML was made. The patient was started with imatinib mesylate 400 mg/day, and within 4 weeks of therapy, his Ann Hematol (2009) 88:89–90 DOI 10.1007/s00277-008-0545-1

Multi-organ failure due to Mycobacterium tuberculosis and Aspergillus flavus infection after allogeneic bone marrow transplantation.

Mycobacterium tuberculosis (MT) is a serious, but rare infectious complication after allogeneic bone marrow transplantation (BMT). We describe a case of fatal sepsis due to MT and Aspergillus flavus after allogeneic BMT for Aplastic Anemia. The diagnosis was made on bone marrow biopsy and asitic fluid culture. Broadspectrum antituberculous and Amphotericin B therapy was started immediately after diagnosis. The patient developed severe hypoxia and finally died of multi-organ failure. Rapid progression of mycobacterial infection as well as fungal infection should be considered in patients post BMT with unexplained fever, particularly in patients from endemic areas.

Efficacy of immunization against hepatitis B virus infection in acute leukemia

OBJECTIVE The aim of this study was to assess the antibody response to combined passive-active immunization versus active immunization against hepatitis B in 71 patients with acute leukemia with negative hepatitis B virus serology at presentation. METHODS The first group (n=28) received a double dose of hepatitis B vaccine at 0, 1, 2 and 6 months and immunoglobin (HBIG) at 0 and 1 month concurrently with vaccine but at a different intramuscular site. The second group (n=43) received double dose of hepatitis B vaccine at 0, 1, 2, and 6 months. HBsAg and anti-HBs titers were determined one month after the 1st, 2nd, 3rd and 4th doses of vaccine. ESULTS In the vaccine-only group, 2.56%, 8.33%, 14.28% and 34.29% of patients developed anti-HBs titer ≥10 IU/L after the 1st, 2nd, 3rd and 4th doses of vaccine, respectively. In the HBIG group, 91.30%, 91.30%, 69.56% and 73.91% of patients developed anti-HBs titer ≥10 IU/L after the 1st, 2nd, 3rd and 4th doses of vaccine, respectively. Those in the vaccine-HBIG group maintained their anti-HBs titer ≥10 IU/L from the 1st to the 4th doses. In the vaccine-only group, 34.29% of patients gained protective antibody titer after receiving the 4th dose of vaccine. Subgroup analysis of age (pediatric vs adult) and disease (acute lymphoblastic leukemia vs acute myeloid leukemia) groups showed no effect of either on the development of protective antibody titer. The incidence of HBsAg positivity one month after the 4th dose of vaccine was 8.62%. No patient became positive for anti-HCV or HIV antibody before or after chemo therapy. CONCLUSION Combined HBIG and vaccine may protect acute leukemia patients during the intensive chemotherapy period.