8 research outputs found
Clinico-biological features and outcome of B- Lymphoblastic Lymphoma (B-LBL) in children
Activity and toxicity of 2-CDA in Langerhans cell histiocytosis: A single institutional experience
Background : Langerhans cell histiocytosis (LCH) is a rare disorder
characterized by clonal proliferation of immature and abnormal bone
marrow derived langerhans cells. Treatment is usually multimodal.
Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and
its proven efficacy in many lymphoproliferative disorders has made
2-CDA a rational choice in treatment of LCH. Aim : To evaluate the
efficacy and toxicity profile of 2-CDA in children with relapsed or
refractory LCH. Setting and Design : This is a pilot study and we
present the initial data of the first seven patients treated at our
institution. Materials and Methods : Seven patients of relapsed and
refractory LCH were enrolled from July 2000 to June 2004. The cohort of
seven patients included six males and one female with a median age at
initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years).
Three patients had received one prior chemotherapy regimen while the
rest were heavily pretreated. Cladribine was administered over two
hours IV daily for five days and repeated every four weeks. Results :
After a median of six courses of cladribine (range, 2 to 9), two (33%)
patients achieved PR and two (33%) patients have SD on imaging but are
clinically better. None experienced grade 3 or 4 hematologic toxicity.
At a median follow-up of 19 months (range, 8 to 52 months), five
patients remain alive and one patient has died. Conclusion : Our study
shows that single agent 2-CDA is active and well-tolerated in children
with relapsed or refractory LCH
Activity and toxicity of 2-CDA in Langerhans cell histiocytosis: A single institutional experience
Background : Langerhans cell histiocytosis (LCH) is a rare disorder
characterized by clonal proliferation of immature and abnormal bone
marrow derived langerhans cells. Treatment is usually multimodal.
Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and
its proven efficacy in many lymphoproliferative disorders has made
2-CDA a rational choice in treatment of LCH. Aim : To evaluate the
efficacy and toxicity profile of 2-CDA in children with relapsed or
refractory LCH. Setting and Design : This is a pilot study and we
present the initial data of the first seven patients treated at our
institution. Materials and Methods : Seven patients of relapsed and
refractory LCH were enrolled from July 2000 to June 2004. The cohort of
seven patients included six males and one female with a median age at
initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years).
Three patients had received one prior chemotherapy regimen while the
rest were heavily pretreated. Cladribine was administered over two
hours IV daily for five days and repeated every four weeks. Results :
After a median of six courses of cladribine (range, 2 to 9), two (33%)
patients achieved PR and two (33%) patients have SD on imaging but are
clinically better. None experienced grade 3 or 4 hematologic toxicity.
At a median follow-up of 19 months (range, 8 to 52 months), five
patients remain alive and one patient has died. Conclusion : Our study
shows that single agent 2-CDA is active and well-tolerated in children
with relapsed or refractory LCH
Clinico-Hematological Profile in Biphenotypic Acute Leukemia
Background : We present a clinico-hematological profile and treatment
outcome of Biphenotypic Acute Leukemia (BAL). Aim : Study incidence
and subtypes of BAL, correlate with age, morphology, and cytogenetic
findings and correlate the clinico-hematological data with the
treatment response. St Jude\u2032s and the EGIL\u2032s criteria have
been compared for their diagnostic and clinical relevance. Material
and Methods : Diagnosis was based on WHO classification, including
clinical details, morphology, cytochemistry, immunophenotyping, and
molecular genetics. We included those cases, which fulfilled the
European Group for the Immunological Characterization of Acute
Leukemia\u2032s (EGIL\u2032s) scoring system criteria for the
diagnosis of BAL, as per recommendation of the WHO classification.
Results : There were 32 patients diagnosed with BAL, based on
EGIL\u2032s criteria. Incidence of BAL was 1.2%. B-Myeloid (14 cases)
followed by T-Myeloid BAL (13 cases) were the commonest subtypes.
Polymorphous population of blasts (16 cases) was commonly associated
with T-Myeloid BAL (10 cases). BCR ABL fusion positivity was a common
cytogenetic abnormality (seven cases). Fifteen patients received
chemotherapy; eight achieved complete remission (CR) at the end of the
induction period. Conclusions : Pediatric BAL and T-B lymphoid BAL
have a better prognosis. A comprehensive panel of reagents is required,
including cytoplasmic markers; to diagnose BAL. St Jude\u2032s
criteria is a simple, easy, and cost-effective method to diagnose BAL.
The outcome-related prognostic factors include age, HLA-DR, CD34
negativity, and subtype of BAL. BCR-ABL expression is an important
prognostic factor, as these cases will be labeled as Chronic myeloid
leukemia (CML) in blast crisis with biphenotypic expression and treated
accordingly
Clinico-Hematological Profile in Biphenotypic Acute Leukemia
Background : We present a clinico-hematological profile and treatment
outcome of Biphenotypic Acute Leukemia (BAL). Aim : Study incidence
and subtypes of BAL, correlate with age, morphology, and cytogenetic
findings and correlate the clinico-hematological data with the
treatment response. St Jude′s and the EGIL′s criteria have
been compared for their diagnostic and clinical relevance. Material
and Methods : Diagnosis was based on WHO classification, including
clinical details, morphology, cytochemistry, immunophenotyping, and
molecular genetics. We included those cases, which fulfilled the
European Group for the Immunological Characterization of Acute
Leukemia′s (EGIL′s) scoring system criteria for the
diagnosis of BAL, as per recommendation of the WHO classification.
Results : There were 32 patients diagnosed with BAL, based on
EGIL′s criteria. Incidence of BAL was 1.2%. B-Myeloid (14 cases)
followed by T-Myeloid BAL (13 cases) were the commonest subtypes.
Polymorphous population of blasts (16 cases) was commonly associated
with T-Myeloid BAL (10 cases). BCR ABL fusion positivity was a common
cytogenetic abnormality (seven cases). Fifteen patients received
chemotherapy; eight achieved complete remission (CR) at the end of the
induction period. Conclusions : Pediatric BAL and T-B lymphoid BAL
have a better prognosis. A comprehensive panel of reagents is required,
including cytoplasmic markers; to diagnose BAL. St Jude′s
criteria is a simple, easy, and cost-effective method to diagnose BAL.
The outcome-related prognostic factors include age, HLA-DR, CD34
negativity, and subtype of BAL. BCR-ABL expression is an important
prognostic factor, as these cases will be labeled as Chronic myeloid
leukemia (CML) in blast crisis with biphenotypic expression and treated
accordingly
Clinico-biologic profile of Langerhans cell histiocytosis: A single institutional study
Context: Langerhans cell histiocytosis (LCH) is a rare atypical
cellular disorder characterized by clonal proliferation of Langerhans
cells leading to myriad clinical presentations and highly variable
outcomes. There is a paucity of Indian studies on this subject. Aim: To
present the experience of management of LCH at a single institution.
Settings and Design: This is a retrospective observational study of
patients with LCH who presented at the Tata Memorial Hospital between
January 1987 and December 2002. Materials and Methods: Fifty-two
patients with LCH were treated in the study period. Due to the long
observation period and variability in diagnostic and therapeutic
protocols, the patients were risk-stratified based on present criteria.
The disease pattern, management approaches and treatment outcomes of
patients were recorded. Statistical Analysis Used: Statistical
analyses were done using Student\u2032s \u2032t\u2032 test, test for
proportion and survival estimates based on the Kaplan-Meier method.
Results: The median age at presentation was 3 years and more than 48%
of the patients had Group I disease. Skeleton, skin and lymphoreticular
system were the commonly involved organs. Majority (80%) required some
form of therapy. The projected overall survival is 63% at 10 years and
mean survival is 118 months. Seventeen percent of surviving patients
developed long-term sequelae. Conclusions: The clinico-biologic
profile of LCH patients in India is largely similar to international
patterns except a higher incidence of lymphoreticular involvement.
Majority of the patients respond favorably to therapy and have a good
outcome, except a subset of Group I patients who warrant enrolment in
clinical trials with innovative therapeutic strategies to improve
outcome