Dharma Choudhary

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.

Hb SE disease: a clinico-hematological profile

Hb S and Hb E are globally common hemoglobinopathies. However, Hb SE double heterozygous state is uncommon, with only 25 cases reported so far in literature. We present two more cases. One presented with gallstones, and the other was asymptomatic. This type of disorder was previously described as a relatively asymptomatic condition compared to HbSS. A review of the 25 reported cases in literature shows that 40.7% (11/27) of these cases are symptomatic. Gender, hematological parameters and levels of Hb S, E or F do not predict clinical severity.

Effect of wheat grass therapy on transfusion requirement in β-thalassemia major

ObjectiveTo assess the efficacy of wheat grass juice on transfusion requirement in patients with beta thalassemia major.MethodsFifty-three patients of thalassemia major with a median age of 16 years were given wheat grass juice tablets.ResultsThe Mean pre and post wheat grass therapy, the pack cell requirement was 288.06 ± 53.25 gm/Kg/year and 301.25 ± 54.86 gm/Kg/year (p =0.054) respectively. No adverse effects were noted.ConclusionWe conclude that wheat grass therapy for one year is not effective in reducing the transfusion requirement in transfusion dependent thalassemia.

Primary myelodysplastic syndrome in children—clinical, hematological and histomorphological profile from a tertiary care centre in India

Abstract We describe the clinical, hematological and histomorphological features in children of primary myelodysplastic syndrome (MDS) seen at the All India Institute of Medical Sciences over three years (Jan 2001–Jan 2004). Twenty-one patients of primary MDS aged 17 year or less were classified using the latest proposed WHO classification for Pediatric MDS. The median age was 9 years with male predominance (80%). Pallor was present in all the cases while fever and bleeding diathesis was present in more than 50% of the cases. Morphological assessment of the peripheral blood showed macrocytosis in 50%, pancytopenia in 15% and blast cells in 45% of cases. A complete analysis of clinical features in conjunction with the bone marrow profile revealed 8 cases of refractory cytopenia (RC), 3 cases of refractory anemia with excess blasts (RAEB), 5 cases of refractory anemia with excess blasts in transformation (RAEB-T), 4 cases of Juvenile myelomonocytic leukemia (JMML) and a solitary cases of acute myeloid leukemia (AML) in Downs syndrome. These children were followed up from 1–36 months (mean 15 months). Three patients of RAEB-T progressed to AML within 3–4 months. RC had the best prognosis and all are alive and under regular follow up. The solitary case of AML of Downs syndrome died 1.5 months after initial diagnosis. All 3 cases of RAEB are under regular follow-up and doing well. Three cases of RAEB-T died (all had progressed to AML); the remaining 2 cases were lost to follow up. Of the 4 cases of JMML 1 died within 6 months of diagnosis; the other 3 cases are under regular follow up of whom 1 has a progressively increasing blast count. We conclude that the latest proposed WHO classification for Pediatric MDS can be successfully applied to all cases of primary MDS.

Comparison of Amicus and COBE Spectra for allogenic peripheral blood stem cell harvest: Study from tertiary care centre in India

INTRODUCTION Most common source of stem cell graft for both autologous and allogenic haematopoietic transplants are peripheral blood haematopoietic progenitor stem cells. Adequate collection of the CD34+ cells and safety of the allogenic donor during the leukapheresis are of prime importance to an apheresis physician. Our retrospective analysis is a comparison between of two platforms namely, COBE Spectra and Amicus, for CD34+ mononuclear cell collection. MATERIAL AND METHOD The study included the data of GSCF (Granulocyte-Colony-Stimulating Factor) mobilized allogenic PBSC collections at our centre from January 2015 to June 2016. The apheresis platforms used were COBE Spectra and Amicus. Blood cell counts were done using LH750 Beckman Coulter (Florida, Miami, USA). CD45+ & CD34+ cell counts were done using BD FACS Canto-II Flow-Cytometer by ISHAGE guidelines. RESULTS A total of 170 PBSC (100 COBE Spectra & 70 Amicus) harvests were done on 143 donors, of which 116 completed the collection in a single session and 27 required a second session. Demographic details and pre harvest peripheral blood counts for both the groups did not show any statistical differences. Amicus processed higher blood volume with higher ACD exposure and procedure time compared to COBE Spectra. Higher platelets loss was with COBE Spectra harvests with higher product volumes collection. Collection efficiency (CE2), collection ratio, CD34+ cells dose was similar on both the platforms. RBC contamination, absolute lymphocyte and monocytes counts were significantly higher with Amicus harvest product compared with COBE Spectra. A total of 14 (8.2%; citrate toxicity) adverse reactions were reported out of 170 allogenic PBSC collections. DISCUSSION/CONCLUSION Our study suggests that both Amicus and COBE Spectra platforms offer comparable results for allogenic PBSC collections. Amicus offers a concentrated PBSC product with lesser volume and platelets loss but higher RBC contamination.

Rota Virus Vaccine Induced Immune Thrombocytopenia

Rota virus is a common cause of diarrhea in infants. Rarely, it has been reported to cause immune thrombocytopenia (ITP). Oral rota virus vaccine has been used to reduce the incidence of rota virus induced diarrhea, however, the vaccine can cause diarrhea. We report here a case of rota virus vaccine induced acute ITP which responded to intravenous immunoglobulins.

Gastrointestinal Biopsies for Evaluation of Acute Graft-Versus-Host Disease in Allogeneic Hematopoietic Stem Cell Transplant Patients

Graft-versus-host disease (GVHD) is the major complication post hematopoeitic stem cell transplantation (HSCT) causing significant morbidity and mortality. Colonic biopsies were performed in 25 post HSCT patients presenting the diarrhea for diagnosis of acute graft versus host disease (A-GVHD). The present study was undertaken to evaluate and illustrate histomorphological features of A-GVHD in GI biopsies and to grade them. Histopathological features of gastrointestinal biopsies from 25 allogeneic HSCT patients having clinical suspicion of A-GVHD were evaluated and compared with colonic biopsies from negative controls. A-GVHD was observed in 17 cases, CMV colitis was present in 3 cases and one case had herpes simplex infection diagnosed in conjunction with serological findings. A-GVHD was graded as grade 1 and 2 in 10 cases and grade 3 and 4 in 7 cases. Apoptosis of crypt epithelial cells was the cardinal feature for diagnosis. Grade 1 and grade 2 A-GVHD cases showed crypt apoptosis in all cases as well as pericryptal apoptosis in lamina propria in many cases. Occasional crypt loss was seen in grade 2 GVHD. Inflammatory infiltrate was composed of lymphocytes and plasma cells. Neutrophils were inconspicuous. Grade 3 and grade 4 A-GVHD cases showed contiguous areas of multiple crypt loss and ulceration with inflammatory infiltrate predominantly composed of lymphocytes and plasma cells, but neutrophils were more prominent than in grade 1 and 2 A-GVHD. Apoptosis of crypt epithelial cells was present in all grade 3 &4 cases except one case. CMV cases were diagnosed by CMV inclusions and IHC stain. Several factors including drug-induced side effects and infections can cause difficulty in histologic interpretation of gastrointestinal biopsies for GVHD. Proper histomorphological interpretation of intestinal A-GVHD is critical for clinical management. A-GVHD is treated with immunosuppression which may worsen infective condition, if present.

Daratumumab (Anti-CD38) interference with serological testing: An emerging challenge for blood banks in developing countries

Daratumumab (DARA), a monoclonal anti-CD38 antibody, belongs to the new generation of immunotherapy in refractory relapsed multiple myeloma. CD38 is weakly expressed on human erythrocytes. By its intrinsic anti-CD38 activity, DARA also interferes in routine pretransfusion compatibility testing such as antibody screening for red blood cells (RBCs) alloantibodies and compatibility testing. Treating RBCs with dithiothreitol eliminates the DARA interference. We report two cases of serological interference of DARA in pretransfusion testing and how timely information before starting the second patient on DARA prevented the delay in pretransfusion compatibility testing and blood availability.

Comparison of “Amicus and COBE Spectra” for autologous peripheral blood stem cell harvest: An Indian experience

Background: Adequate collection of peripheral stem cells from the patients depends on the disease condition, efficient mobilization and the equipment used for PBSC harvest. COBE Spectra has been the major platform for collecting these PBSC for more than 2 decades. Now with introduction of PBSC harvest option with Amicus cell separators (which was used primarily for plateletpheresis) in India, it is worth while comparing both the platforms for stem cells collections. Materials and Methods: Our study is a retrospective analysis of autologous PBSC harvest procedures done at our centre. The study included the data of autologous PBSC collections from January 2015 to June 2016. Total 61 patients underwent 85 autologous PBSC harvests for both haematological and non haematological indications. Results: Out 61 patients, 40 patients collected their target number of cell in a single harvest, 18 patient required dual harvests and 3 patients required three consecutive days of harvest. Pre-Apheresis WBC, platelets and CD45/CD34 cell counts were comparable. COBE Spectra collects significantly higher product volume and higher number of platelets in the apheresis product. Whereas WBC counts of the product, total CD45/CD34 cell dose and collection efficiency (CE2) and collection ratio (CR) were comparable on both the platforms. Conclusions: Amicus took more time to harvest the anticipated number of cells in the graft as well as COBE Spectra resulted in higher platelets loss during the process of cell collection. Our analysis is first of its kind from Indian subcontinent and indicates that with gradual phasing out of COBE spectra from the market Amicus offers a comparative platform for PBSC harvest.

Hematopoietic Stem Cell Transplant in Elderly Patients: Experience from a Tertiary Care Centre in Northern India

One-fifth of the world’s population resides in India. The burden of hematological diseases both malignant and nonmalignant is huge in the country. According to the registry data the total number of leukemia and lymphoma patients living in India in 2010 was about 100,000 [1]. The estimated size of the elderly population in India is expected to rise from 77 million in 2001 to 140 million by 2021. With the increasing awareness about hematological diseases and the rising economy, many patients are opting for hematopoietic stem cell transplant (HSCT) as a definite treatment for many hematological diseases. The role of HSCT for various hematological diseases in young patients is well established. Older patients have traditionally been considered ineligible for stem cell transplantation and data of HSCT in elderly is scarce, particularly from developing countries. Here we report our experience with stem cell transplantation in the elderly population in India. We reviewed the transplant database at Bone Marrow Transplant (BMT) centre, BLK Superspeciality Hospital, New Delhi. The conditioning regimen for allogeneic HSCT was reduced intensity conditioning (with fludarabine 30 mg/m for 5 days and melphalan 140 mg/m for 1 day). For autologous HSCT for multiple myeloma and lymphoma, melphalan 200 mg/m and BEAM regimens (BCNU 300 mg/m, etoposide 200 mg/m, cytarabine 200 mg/m and melphalan 200 mg/m) respectively were used as conditioning regimens. The transplants were performed in High Efficiency Particulate Air (HEPA) filtered rooms. Peripheral blood stem cell harvest was done after granulocyte-colony stimulating factor (G-CSF) mobilization. Graft versus host disease (GVHD) prophylaxis for allogeneic HSCT included cyclosporine and methotrexate. Patients received standard anti-viral prophylaxis with acyclovir and Pneumocystis jiroveci prophylaxis with trimethoprim-sulfamethoxazole. Patients were treated with broad spectrum antibiotics at the time of their first neutropenic fever, and with antifungal agents as per institutional policy. The study was approved by the Institutional Review Board. Out of 440 patients who underwent HSCT at BLK Superspeciality hospital from Feburary 2010 to August 2015, 19 (4.32 %) were C60 years of age. Median age was 63 years (range 60–73 years). There were 14 males and 5 females. Seven patients underwent allogeneic HSCT and 12 patients underwent autologous HSCT. All patients had normal liver and kidney functions and normal left ventricular ejection functions prior to HSCT. Six patients had diabetes well controlled with oral hypoglycemic agents. All allogeneic HSCTs were sibling matched peripheral blood stem cell transplantations. The most common indications for allogeneic and autologous HSCT were acute myeloid leukemia (AML) and multiple myeloma (MM) respectively (Table 1). The donors for allogeneic HSCT were females in five cases and males in two cases with a median age of 56 years (range 51–62 years). The median CD34? stem cell dose was 3.7 9 10/kg recipient body weight (range 1.88–8.77 9 10/kg). Neutrophil engraftment occurred at a median of 11 days (range 9–13 days) and platelet engraftment at 15 days (range 11–26 days). None of the patients had cytomegalovirus (CMV) reactivation. One patient developed grade 2 acute gut GVHD and three patients had limited chronic skin GVHD. Twelve patients & Sanjeev Kumar Sharma sksanjeev13@yahoo.com