Rahul Naithani

Intracranial hemorrhage in childhood immune thrombocytopenic purpura

We retrospectively analyzed 750 patients with ITP for development of intracranial hemorrhage (ICH). Seventeen cases with age range of 10 months to 18 years were studied. Ten patients were of acute ITP and seven had chronic ITP. Nine patients developed ICH one month after the onset of ITP and five patients had ICH on presentation. ICH was precipitated by trauma in four patients and possibly the use of NSAIDs in one patient. Median platelets counts at the time of ICH were 12 × 109/L (range 2–50 × 109/L). Most patients were treated with corticosteroids. Four patients (24%) died due to ICH. Pediatr Blood Cancer 2009;52:529–531.

Thiopurine S-methyltransferase gene polymorphism and 6-mercaptopurine dose intensity in Indian children with acute lymphoblastic leukemia

The prevalence of thiopurine S-methyltransferase (TPMT) polymorphism and its association with clinical and hematological toxicities was retrospectively analyzed in 71 Indian children with acute lymphoblastic leukemia (ALL). Only heterozygous TPMT alleles were observed (10%, 7/71) with relative frequencies being *1/*3C (4.2%), *1/*2 (4.2%) and *1/*3A (1.4%). The median 6-mercaptopurine dose administered during the maintenance therapy was 31% lower among patients with heterozygous TPMT alleles versus the rest (32.1mg/m(2)/day and 46.2mg/m(2)/day, p=0.005), though the myelosuppression and toxicities were similar in both the groups. Identification of TPMT genotype appears to be important in making the ALL treatment more effective and less toxic.

Efficacy of cyclosporine as a single agent therapy in chronic idiopathic thrombocytopenic purpura

We read with interest the letter by Emilla et al. [1][1] describing long-term results of low dose cyclosporine in idiopathic thrombocytopenic purpura (ITP). It would have been interesting to know in how many patients was cyclosporine used so that the overall response rates could be known. There are

Increased complications and morbidity in children with hemophagocytic lymphohistiocytosis undergoing hematopoietic stem cell transplantation.

Hematopoietic stem cell transplantation (HSCT) is the only curative option for patients with primary hemophagocytic lymphohistiocytosis (HLH) and for patients with secondary HLH who fail to respond to therapy.

CD20 has no prognostic significance in children with precursor B-cell acute lymphoblastic leukemia

Acute lymphoblastic leukemia (ALL) is the most common leukemia in children. Survival outcomes have greatly improved with combination chemotherapy and the intensification of treatment.1 Recent improvements in ALL management have largely been due to optimizing treatment according to various risk stratification schema and evaluation of minimal residual disease (MRD) at the end of induction treatment has proved valuable.2 However, up to half of relapse events occur in patients who are MRD negative at end-induction.3 We read with interest the article by Mannelli et al.4 and examined whether CD 20 was an adverse prognostic marker in children with precursor B-ALL. We carried out a retrospective analysis of children (aged 1–18 years) diagnosed with precursor B-ALL over six years between 1st January 2004 to 31st December 2009. The study was approved by the local institutional review board. We considered 20% to be the threshold for positivity (CD20+). Standard risk patients were treated with either POG 9904, POG 9905 or AALL0331, and high-risk patients were treated with either POG 9906 or AALL0232. MRD analysis was made using flow cytometry. MRD values of more than 0.1% were considered positive. Data were analyzed using SPSS software version 11.5. Survival outcomes were assessed by the Kaplan-Meier method and log rank test.5 Data analysis was censored on 31st December 2010. A total of 326 children were diagnosed with ALL over the 6-year period including 259 children treated for precursor B-ALL. Patients’ characteristics are shown in Table 1. Mean age of the study cohort was 5.2±4.0 years and median presenting WBC count was 9.1×109/L (1–841×109/L). Six patients had end of induction MRD between 0.1 to 1% and 2 had more than 1%. Fourteen patients had an event (13 relapses, 4 deaths). Mean follow up was 41 months ±20 months (median 41 months). There were 2 deaths in each of the CD 20 positive and negative groups. Table 1. Patients’ characteristics. Overall survival was 98% and this was similar in both groups. Event free survival (EFS) of the standard and the high-risk groups according to CD 20 status is shown in Figure 1A–C. Altogether, CD 20 did not indiate poor prognosis in this population. Results were similar when children with positive CD 20 were divided into those with more than 20% or those with more than 40% positivity. MRD at Day 29 was the strongest predictor of both death (P=0.00) and relapse (P=0.034). Figure 1D shows event free survival in the MRD negative group according to CD 20 status. Figure 1. (A) Event free survival in all children according to CD 20 status. (B) Event free survival in standard risk group according to CD20 status

High dose dexamethasone therapy shows better responses in acute immune thrombocytopenia than in chronic immune thrombocytopenia

Oral high dose dexamethasone (HDD) was given as a single daily dose for four consecutive days, every 14 days for four courses. Twenty-nine patients were enrolled. Overall 20 patients (69%) responded: complete response (CR) was achieved in 16 (55%) patients, partial response (PR) in three (10%) patients and MR in one (3%) patient. In acute immune thrombocytopenic purpura (ITP) response rates after the first, second, third and fourth cycles were as follows: 64% (9/14), 64% (9/14), 79% (11/14), and 85.7% (12/14), respectively. In chronic ITP, overall response rates after the first, second, third and fourth cycles were as follows: 33% (5/15), 40% (6/15), 53% (8/15) and 53% (8/15) respectively. The median time to response was 14 days (4–42 days). Twelve out of 20 patients (5/12 acute ITP and 7/8 chronic ITP) relapsed; median relapse free survival till last follow-up in the remaining eight patients was 130 days (65–365 days).

Early discharge from hospital after consolidation chemotherapy in acute myeloid leukemia in remission: febrile neutropenic episodes and their outcome in a resource poor setting

Treatment of acute myeloid leukemia (AML) involves administration of myelosuppressive chemotherapy administered after admittance to hospital.[1][1] Admission to intensive nursing care units till bone marrow recovery leads to prolonged hospital stay. Quality of life and health care issues have made

Allogeneic cord hematopoietic stem cell transplantation in an infant with primary myelofibrosis.

Primary myelofibrosis (PMF) is rare in children. An allogeneic hematopoietic stem cell transplantation (HSCT) is the only known curative therapy for severe cases. Here, we report the case of a female infant with PMF treated with allogeneic HSCT using an unrelated cord blood unit. She had successful reversal of her disease, but experienced complications related to transplant. This is the seventh reported case of HSCT for PMF in children, and the second using umbilical cord blood. We conclude that cord HSCT is a useful curative treatment option in children with PMF, but that efforts must be taken to reduce complications.

Single Versus Dual Platform Analysis for Hematopoietic Stem Cell Enumeration Using ISHAGE Protocol

Hematopoietic stem cell transplantation is curative therapy in benign and malignant diseases. Adequate stem cell dose is one of the most important marker of engraftment. Several methods have been developed to enumerate CD34+ cells. We present our data on 147 samples analysis. There was a clear linear correlation between two methods. Both methods were effective. Both single vs dual platform analysis yield similar results. Single platform analysis is easier to perform. In terms of cost reduction dual platform analysis is better.

Efficacy and safety of anti-D for treatment of adults with immune thrombocytopenia.

This study was planned to assess the response of anti-D in patients with immune thrombocytopenia (ITP). Twenty adults (8 males: 12 females) with a median age 33.5 years (19–59 years) were included. Nine patients had newly-diagnosed ITP, 6 had persistent ITP and 5 had chronic ITP. The overall response rate was 65%. Patients with newly diagnosed ITP showed response rates of 77% (7/9), persistent ITP had response rates 50% (3/6) and patients with chronic ITP had response rates of 60% (3/5). The median time to response was 3 days (1–11 days). There was no correlation of response with age, sex, severity of bleeding, presenting platelet counts, ABO blood group or prior steroid or IVIG response.