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Table of Contents
April-June 2016
Volume 7 | Issue 2
Page Nos. 47-82
Online since Thursday, July 14, 2016
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REVIEW ARTICLE
Assessment of minimal residual disease in childhood acute lymphoblastic leukemia
p. 47
Sonia K Parikh, Urmila P Uparkar
DOI
:10.4103/1658-5127.186323
The improving cure rate of childhood acute lymphoblastic leukemia (cALL) is considered as success story in the field of oncology. It has become possible due to progressive refinement of treatment over the period of years. Assessment of minimal residual disease (MRD) is one such tool to further refine and personalize treatment of cALL. Assessment of MRD is no longer a research tool; it has become an integral part of comprehensive management of cALL. Prognostic importance of MRD in cALL is well accepted, but translation of this new information in improving therapy has just begun especially in developing countries like ours. There is increasing understanding among clinicians regarding importance of MRD assessment in day to day clinical practice. Still, there are many issues and lack of clarity with respect to MRD assessment. We are making an attempt in this review to address such issues faced during practical implication of MRD assessment.
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ORIGINAL ARTICLES
Stem Cell Transplantation in children: A single-center experience in Saudi Arabia
p. 54
Muhammad Matloob Alam, Ibraheem Abosoudah, Shara Al Harbi, Marwa Elhadidy, Mohamed Bayoumy
DOI
:10.4103/1658-5127.186324
Introduction:
Bone marrow transplantation (BMT) has frequently considered as a curative treatment for children with serious hematological and malignant disorders. The aim of this study was to determine the indication, frequencies of transplant-related morbidity, and outcome at our tertiary health care facility.
Materials and Methods:
We retrospectively analyzed the clinical, laboratory, and outcome data of all 131 pediatric patients consecutively underwent BMT between 2005 and 2014. SPSS package software version 20.0 (IBM, Chicago, IL, USA) was used for statistical analysis and Kaplan-Meier curve were constructed for overall survival (OS) and event free survival (EFS), and compared according to type of BMT, impact of graft-versus-host disease (GVHD), and cytomegalovirus (CMV) reactivation on survival function were evaluated.
Results:
The mean age of the study population at the time of transplant was 6.5 ± 4 years. Eighty-five (64.9%) were males and 46 (35.1%) were females. Majority of patients had nonmalignant hematological disorder (
n
= 51, 38.2%) followed by hematological malignancy (
n
= 50; 38.2%) and solid tumors (
n
= 30; 22.8%). Most of the patients received allogeneic transplant (
n
= 92; 70.2%) and remaining received autologous transplant (
n
= 39; 29.8%). Acute and chronic GVHD was observed in 26 (19.8%) and (6.9%) cases, respectively. Most of the patients were engrafted (
n
= 109; 83.2%). CMV reactivation was observed in 38 patients (29%); out of them majority were asymptomatic (
n
= 35/38; 92.1%) and in most of cases CMV were resolved (
n
= 35/38; 92.1%) with antigenemia-guided preemptive therapy with ganciclovir. OS and EFS rate were 69.5% and 59.6%, respectively. OS time was 35.5 months and EFS time was 31.6 months with median duration of follow-up of 72 months.
Conclusions:
The results of the pediatric BMT program at our institution have been comparable to those reported in the literature as far as transplant-related morbidity and mortality is concerned.
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"Not so benign"
Plasmodium vivax
malaria: An appraisal
p. 63
Chandrika Rao, Karuna Ramesh Kumar
DOI
:10.4103/1658-5127.186325
Introduction:
Plasmodium
vivax
infection-associated hematological changes including severe anemia can cause significant morbidity and indirect mortality.
Objective:
The objective of the study is to analyze hematological changes in
P. vivax
malaria patients and to review pathogenesis and cause of death in
P. vivax
infection.
Materials and Methods:
During a 1-year period, patients who were screened and confirmed as
P. vivax
by quantitative buffy coat and peripheral smear were included in the study. The demographic details and hematological profile (hemoglobin, total white blood cell count, and platelet count) were correlated. The severity of anemia was graded based on hemoglobin and correlated with parasitemia.
Results:
Two hundred patients were included. Twelve of them in addition showed
Plasmodium
falciparum
. Thrombocytopenia (74.5%) was a significant feature compared to anemia (4.5%) and leukopenia (20.5%). Only three patients had hemoglobin level <6 g%. One of them (4.3 g%) developed relapse. During relapse, he had hemolytic anemia which required transfusion. He died on follow-up. All patients were treated with the first-line antimalarial drugs.
Conclusions:
In view of the inherent biological characteristics,
P. vivax
is more difficult to eradicate than
P. falciparum.
The pathogenesis of anemia is multifactorial including mechanical processes and immune mechanisms which enhance removal of infected and uninfected cells. This probably leads to severe anemia in the patient who died.
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A case series of patients with
de novo
Philadelphia-positive acute myeloid leukemia at a tertiary care center in South India - clinical profile, outcomes, and review of literature
p. 66
D Loknatha, Suparna Ajit Rao, KN Lokesh, K Govind Babu, MC Suresh Babu, KC Lakshmaiah
DOI
:10.4103/1658-5127.186326
Introduction:
Ph+ AML is a well-recognized , but rare entity that poses challenges to clinicians to differentiate it from Chronic myeloid leukemia in blast crisis.
Objective:
Our aim was to study the clinical profile, treatment and outcomes of patients with Ph+ AML at our centre.
Materials and Methods:
Four cases of Ph+ AML entered in the hospital registry between 2013 and 2015 were identified and analysed.
Results:
The median age was 46 years with equal sex preponderance. The duration of presenting complaints was <3 months. Median presenting total count was 54,000 cells/cumm. 3 patients had mild splenomegaly. None had basophilia. There was no predominant FAB-subtype. The cytogenetics revealed abnormalities in addition to the Philadelphia chromosome in 2 patients.3 out of 4 patients received induction chemotherapy with 3+7 - cytosine arabinoside and daunomycin alon with Imatinib dosed at 400 mg/ day. They were all in hematological, morphological and cytogenetic remission following induction. None underwent allogenic stem cell transplant. Of the 4, one refused treatment and is lost to follow up. Of those who received induction chemotherapy, 2 received consolidation with high dose ARAC, of which- one completed consolidation (4 courses) and is in remission 27 months after diagnosis, and the other has completed 1st high dose consolidation (and is alive 3 months after diagnosis) and the third one received 3 courses of low dose subcutaneous ARAC due to poor tolerability and expired 7 months after diagnosis.
Conclusions:
Ph+ AML is rare with no clear treatment recommendations. Improved outcomes with tyrosine kinase inhibitors(TKI's) and stem cell transplant should prompt further studies. If patients remain in remission with long term TKI use, it could serve as an alternative to stem cell transplantation in low income countries.
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CASE REPORTS
Aleukemic granulocytic sarcoma and leukemia cutis: A report of two rare cases and review of literature
p. 70
Rahul S Kulkarni, Asha S Anand, Apurva A Patel, Sandip A Shah, Harsha P Panchal, Sonia K Parikh, Hemangini H Vora, Biren P Parikh, Avinash Talele
DOI
:10.4103/1658-5127.186327
Granulocytic sarcoma (GS), also called myeloid sarcoma is an extramedullary tumor of the immature granulocytic cells. It is a rare entity and mostly accompanied by acute myeloid leukemia (AML). Very rarely, it is detected before clinical signs of leukemia or other diseases. When the bone marrow biopsy reveals no other hematologic malignancies, the GS is described as aleukemic, primary or isolated. Here, we report two rare cases, one of which presented as aleukemic GS of lymph nodes with aleukemic leukemia cutis, and the other with aleukemic GS of lung. Both cases posed diagnostic dilemma in view of their atypical presentations and site of involvement. Final diagnosis was made by immunohistochemistry (IHC). Both patients were treated with standard induction chemotherapy for AML. One patient had relapsed on treatment and was further treated with only 6-thioguanine leading to complete remission. Our cases emphasize the importance of early suspicion and use of IHC in diagnosis of aleukemic GS and also potential role of oral thioguanine alone in relapsed cases not eligible for hematopoietic stem cell transplant.
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Acute intravascular hemolysis following accidental ingestion of naphthalene: A rare case report
p. 76
Rachita Sarangi, Sarita Pradhan, Bikrant Kumar Prusty, Sitaram Mahapatra
DOI
:10.4103/1658-5127.186328
Naphthalene toxicity, an unusual form of poisoning, is often missed as a probable diagnosis for acute onset of intravascular hemolysis in children. It has an unpredictable clinical course and proper management in children relies heavily on high index of clinical suspicion. We report a rare case of naphthalene toxicity in a 1½-year-old child who presented with acute onset of intravascular hemolysis following accidental ingestion of a single mothball. She was put on supportive therapy. We have reported the present case to highlight the spectrum of acute symptoms that a single accidental ingestion of a moth ball can cause. The idiosyncrasy of the chemical toxicity and the myriad of symptoms that it can cause has to be kept in mind to impart timely and right treatment to save precious human lives.
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LETTER TO EDITOR
Severe eosinophilia with relative neutropenia associated with low dose clozapine therapy
p. 79
Erika Pahuja, Jyoti Singh, Sujita Kumar Kar, Amit Singh
DOI
:10.4103/1658-5127.186329
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IMAGE IN HEMATOLOGY
Bone marrow tuberculosis: A diagnosis not to be missed
p. 81
Nadeem Tanveer, Kavana Soni
DOI
:10.4103/1658-5127.186330
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