Publications by authors named "Shano Naseem"

88 Publications

Chromosome 7q31 Amplification in Fluorescence Hybridization: A Hallmark of Hepatosplenic Gamma Delta T Cell Lymphoma.

Indian J Hematol Blood Transfus 2021 Jul 13;37(3):514-516. Epub 2020 Nov 13.

Department of Hematopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

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http://dx.doi.org/10.1007/s12288-020-01380-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8239067PMC
July 2021

Myeloid Neoplasm with t(8;22)(p11;q11): A Mimicker of Chronic Myeloid Leukaemia in Blast Crisis.

Indian J Hematol Blood Transfus 2021 Apr 31;37(2):334-336. Epub 2020 Aug 31.

Department of Hematopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

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http://dx.doi.org/10.1007/s12288-020-01343-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8012448PMC
April 2021

Efficacy, safety, and quality of life of generic and innovator ibrutinib in Indian CLL patients.

Indian J Hematol Blood Transfus 2021 Apr 13;37(2):313-317. Epub 2020 Nov 13.

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

To report the efficacy, safety, and quality of life (QoL) on generic and innovator ibrutinib in Indian CLL patients. This was a single centre, prospective study of treatment-naive (TN), and relapsed/refractory (R/R) CLL patients receiving ibrutinib in India. The choice of innovator or generic ibrutinib was as per patient discretion. Response and adverse events were recorded as per the 2018 iwCLL guidelines and CTCAEv4.0. QoL was assessed using the EORTC QLQ-C30 and CLL17 questionnaires. A total of 32 CLL patients (TN, n = 7 and R/R, n = 25) received ibrutinib from 2016-2019. The median age was 60 years (37-84). All TN patients attained partial response without any grade 3/4 adverse events (AE). Ibrutinib was less tolerated in the R/R setting, with 52% patients developing grade 3/4 AE and required dose reduction. Eleven patients (44%) died during follow-up. Grade 3-5 infections were seen in 44% of R/R CLL patients. Generic ibrutinib (n = 8) was comparable to innovator ibrutinib (n = 17) in terms of efficacy, safety, and QoL. Ibrutinib is less well tolerated in Indian R/R CLL patients. Infections are a common cause of morbidity and mortality. This study affirms the safety and efficacy of generic ibrutinib.
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http://dx.doi.org/10.1007/s12288-020-01378-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8012457PMC
April 2021

and Gene Mutations in Acute Myeloid Leukemia.

Int J Hematol Oncol Stem Cell Res 2021 Jan;15(1):15-26

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

A number of mutations have been reported to occur in patients with acute myeloid leukemia (AML), of which and genes mutations are the commonest and have important diagnostic and therapeutic implications. Molecular testing for and genes was performed in 92 de-novo AML patients. The frequency and characteristics of and mutations were analyzed. and mutations were seen in 22.8% and 16.3% of patients, respectively. Amongst mutations, -ITD mutation was seen in 8.7% cases, -TKD in 5.4%, and -ITD+TKD in 2.2% cases. Certain associations between the gene mutations and clinical characteristics were found, including in mutated group- female preponderance, higher incidence in M4/M5 categories and decreased expression of CD34 and HLA-DR; and in -ITD mutated group- higher age of presentation, higher total leucocyte count and blast percentage. - AML patients with and mutations have differences in clinical and hematological features, which might represent their different molecular mechanism in leukemogenesis. The frequency of and mutations in this study was comparable to reports from Asian countries but lower than that reported from western countries. However, as the number of patients in the study was less, a larger number of patients need to be studied to corroborate these findings.
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http://dx.doi.org/10.18502/ijhoscr.v15i1.5246DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7885130PMC
January 2021

Randomized controlled trial of individualized, low dose, fixed duration lenalidomide maintenance versus observation after frontline chemo-immunotherapy in CLL.

Leuk Lymphoma 2021 07 21;62(7):1674-1681. Epub 2021 Feb 21.

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Lenalidomide maintenance after frontline chemo-immunotherapy (CIT) in chronic lymphocytic leukemia (CLL) has not been standard due to the availability of novel therapies, though these remain out of reach for most in low-middle income countries. This single-center, open-label study randomized CLL patients (non-deletion 17p) after frontline therapy to lenalidomide maintenance (dose-escalated 2.5-10mg, 20/28 days per cycle for six months) or observation (2:1 allocation). Forty patients were included over 2018-2020. At a median follow-up of 22 months, median progression-free survival (PFS) with lenalidomide was not significantly different than observation (26 vs. 18 months,  = 0.4). Patients with minimal residual disease >10 had a trend toward better PFS with lenalidomide (19 vs. 7 months,  = 0.07). Grade 3 neutropenia was seen in 16.7% of patients on lenalidomide. Quality of life was comparable between the two arms. Low dose, fixed duration lenalidomide maintenance is not an effective strategy after frontline CIT in CLL.
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http://dx.doi.org/10.1080/10428194.2021.1885668DOI Listing
July 2021

Unusual morphological abnormality of neutrophils in a patient with SARS-CoV-2 infection.

Br J Haematol 2021 05 17;193(4):690. Epub 2021 Feb 17.

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1111/bjh.17355DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8013899PMC
May 2021

Diagnostic performance of biomarkers in maternal sepsis: A prospective observational study.

Int J Gynaecol Obstet 2021 Aug 11;154(2):312-317. Epub 2021 Feb 11.

Department of Hematology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Objectives: Maternal sepsis is a life-threatening condition. Biomarkers have been found to be useful in early detection of sepsis in the critical care setting. We aimed to determine the diagnostic performance of different biomarkers such as procalcitonin, C-reactive protein (CRP), absolute eosinophil count, and activated partial thromboplastin time (aPTT) in maternal sepsis.

Methods: A total of 35 patients were enrolled in this prospective observational study. Patients with suspected sepsis were evaluated for multi-organ dysfunction. The blood samples for testing of these biomarker levels were obtained at the time of enrollment in the study (day 1), and on day 3 and day 7. Trends of each marker were followed and correlated with the clinical picture.

Results: Of 35 enrolled patients, 30 completed the study. Among these, 18 had sepsis and 12 were designated as without sepsis. Sensitivities of procalcitonin, CRP, aPTT, and absolute eosinophil count were 83.33%, 77.78%, 55.56%, and 58.82% whereas their specificities were 66.67%, 75.0%, 100%, and 75%, respectively. Area under the curve was highest for procalcitonin (0.813) followed in decreasing order by CRP (0.778), aPTT (0.731), and eosinophil count (0.642), respectively.

Conclusion: Procalcitonin and CRP may be used as a valuable adjunct in the clinical stepwise approach for the prompt diagnosis of maternal sepsis.
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http://dx.doi.org/10.1002/ijgo.13525DOI Listing
August 2021

Frequency of MYD88 L256P mutation and its correlation with clinico-hematological profile in mature B-cell neoplasm.

Hematol Oncol Stem Cell Ther 2020 Nov 13. Epub 2020 Nov 13.

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Objective/background: B-cell neoplasms are clonal tumors of B cells at various stages of maturation, including diffuse large B-cell lymphoma (DLBCL), chronic lymphocytic lymphoma (CLL), Burkitt lymphoma (BL), lymphoplasmacytic lymphoma (LPL)/Waldenström's macroglobulinemia (WM), splenic marginal zone lymphoma (SMZL), nodal marginal zone lymphoma (NMZL), mantle cell lymphoma (MCL), follicular lymphoma (FL), and hairy cell leukemia (HCL). In this study, we analyzed the frequency of MYD88 L265P mutation and its correlation with clinico-hematological profile in mature B-cell neoplasms.

Methods: A total of 110 consecutive cases of B-cell neoplasms showing peripheral blood and/or bone marrow infiltration were included. MYD88 L265P mutation was detected by polymerase chain reaction amplification of exon 5 of MYD88 gene, followed by restriction fragment length polymorphism analysis.

Results: Among the 110 cases, the major group was of CLL (54.5%, n = 60), followed by HCL. Other cases included MCL, LPL, DLBCL, SMZL, NMZL, FL, and BL. MYD88 L265P mutation was seen in 21 (19.1%) cases of B-cell neoplasm, whereas 89 (80.9%) cases were negative for MYD88 L265P mutation. It was most commonly seen in LPL/WM cases followed by HCL, SMZL, CLL, and MCL cases. No case of DLBCL, FL, and BL showed MYD88 L265P mutation. Statistically significant difference was seen for hemoglobin level in CLL cases, with MYD88 L265P mutated cases showing higher mean hemoglobin levels than MYD88 wild-type cases (p = .001). For other parameters, no statistically significant difference was noted between mutated and unmutated cases.

Conclusion: MYD88 L265P mutation is seen in various B-cell neoplasms; it is most commonly seen in LPL/WM cases but not specific for it.
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http://dx.doi.org/10.1016/j.hemonc.2020.10.003DOI Listing
November 2020

JAK2V617F Mutation in Patient with Splanchnic Vein Thrombosis.

Indian J Hematol Blood Transfus 2020 Oct 25;36(4):700-704. Epub 2020 May 25.

Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh, 160012 India.

Splanchnic vein thrombosis is an uncommon life-threatening form of venous thrombosis. It is one the common complication among MPN's. In the western studies the prevalence of JAK2V617F mutation among SVT patient is high and ranges from 7 to 59%. The frequency of this mutation among Indian SVT patients is heterogenous. This was a prospective case control study. A total 52 cases of SVT and 40 controls were screened for JAK2V617F mutation along with other routine thrombophilic risk factors. Out of total 52 cases, 10 had BCS, 2 had MVT and rest 40 were of PVT/EHPVO. The JAK2V617F mutation was seen in two cases and not in controls. Among the thrombophilic markers, heterozygous FVL mutation, PC, PS and presence of APA were seen in 2, 3, 1 and 3 cases respectively. In addition, eight cases also showed deranged risk factors (5 inherited and 3 acquired), however the repeat testing was not performed due to loss of follow up. Among controls, one person showed presence of APA and one person showed multiple thrombophilic risk factor deficiency. JAK2V617F mutation was observed in 3.8% among north Indian SVT patients. The frequency of mutation is on the lower side as compared to the available Indian data. The other thrombophilia markers (both inherited and acquired) are more frequent (18%) and patients should be routinely screened for these thrombophilia markers.
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http://dx.doi.org/10.1007/s12288-020-01292-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7573011PMC
October 2020

An Evaluation of a Fluorescence In Situ Hybridization Strategy Using Air-dried Blood and Bone-marrow Smears in the Risk Stratification of Pediatric B-Lineage Acute Lymphoblastic Leukemia in Resource-limited Settings.

J Pediatr Hematol Oncol 2021 05;43(4):e481-e485

Unit of Paediatric Haemato-oncology, Department of Paediatrics, Advanced Paediatric Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Cytogenetic abnormalities (CAs), one of the strongest influencers of therapeutic outcome in pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL), can be identified by different techniques. Despite several technological advances, many centers with resource-limited settings continue to use either reverse-transcriptase polymerase chain reaction (RT-PCR) and/or fluorescence in situ hybridization (FISH) to identify prognostically relevant CAs. We evaluated a simple and cost-effective triple-probe FISH strategy on air-dried blood and bone-marrow smears and compared its performance with a multiplex RT-PCR-based approach in the prognostication of pediatric BCP-ALL patients. Three hundred twenty BCP-ALL patients were tested prospectively and in parallel by FISH on air-dried blood or bone-marrow smears and RT-PCR. The FISH strategy correctly diagnosed all genetic abnormalities identified by RT-PCR. Prognostically relevant genetic abnormalities were missed by RT-PCR in 24 (8.1%) patients. In another 20 children (6%), with samples inadequate for RT-PCR testing (dry taps or due to poor sample quality), a successful FISH testing could be performed on bone-marrow aspirate or trephine-imprint smears. In addition, FISH detected ploidy changes, which could be confirmed by FxCycle Violet-based flow-cytometry. FISH testing on air-dried smears identified more prognostically relevant CAs, provided information on the ploidy status, and could be successfully performed in children with difficulty in bone-marrow sampling.
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http://dx.doi.org/10.1097/MPH.0000000000001892DOI Listing
May 2021

Genomic alterations in chronic lymphocytic leukemia and their correlation with clinico-hematological parameters and disease progression.

Blood Res 2020 Sep;55(3):131-138

Departments of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Background: Chronic lymphocytic leukemia (CLL) is a heterogeneous disease, which is attributed to differences in the genetic characteristics of the leukemic clone. We studied the genomic profile of 52 treatment-naïve CLL patients.

Methods: Genetic analysis was performed by multiplex ligation-dependent probe amplification (MLPA) using the SALSA P038 Probemix (MRC Holland, Amsterdam), which contains probes for 2p (), 6q, 8p (), 8q (), 9p21 (), 10q (), 11q (), chromosome 12, 13q14 (), 14q, 17p () and chromosome 19, and for NOTCH1 7541-7542delCT, SF3B1 K700E, and MYD88 L265P mutations.

Results: The median age was 65 years (male:female=2:1). The median hemoglobin, total leukocyte, and platelet counts were 12.4 g/dL, 57.7×10/L, and 176.5×10/L, respectively. At least one genetic abnormality was observed in 34 (65%) patients. The most common abnormality was del(13q14) (deleted and / genes), which was observed in 22 (42%) cases, followed by trisomy 12 [7 (13%) cases]. Del(11q) (deleted ) and del(17p) (deleted ) were present in 5 (10%) and 2 (4%) cases, respectively. 19p13.2 () amplification and mutation were found in one case each.

Conclusion: Genetic abnormalities are commonly (65%) observed in CLL patients. Del(13q), which is associated with and / gene deletion, was the most common. Compared with other abnormalities, del(11q) and del(17p) patients presented with cytopenia and higher Binet stage, while those with del(13q14) had a longer time to first treatment.
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http://dx.doi.org/10.5045/br.2020.2020080DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7536571PMC
September 2020

Chronic Lymphocytic Leukemia: Real-World Data From India.

JCO Glob Oncol 2020 06;6:866-872

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Purpose: Chronic lymphocytic leukemia (CLL) is uncommon in India. There are limited studies on CLL from the Indian subcontinent.

Methods: This was a prospective study (2011-2017) of consecutively diagnosed patients with CLL at a single center. The diagnosis, prognosis, treatment indication, response criteria, and adverse events were recorded as per International Workshop on Chronic Lymphocytic Leukemia guidelines. Biosimilar rituximab dosing (375 mg/m) was fixed for all cycles. Time to next treatment (TTNT) was defined as the time from front-line treatment initiation to next treatment or death from any cause. Overall survival (OS) was defined as the time from treatment initiation until death from any cause.

Results: A total of 409 patients with CLL were enrolled over the study period. The median follow-up was 32 months (range, 2-135 months). The median age was 61 years, and 31.8% of patients with CLL were ≤ 55 years of age; 43.3% of patients had a cumulative illness rating scale score ≥ 3. Prognostic fluorescence in situ hybridization data were available in 53.3% of patients. Chlorambucil (94/180; 52.2%) and bendamustine + rituximab (BR; 57/180; 31.6%) were the most common regimens used up front. The overall response rates after front-line therapy were 74.4% and 91.2%, respectively. The TTNT was 33 months and not reached, respectively ( = .001). Grade 3/4 neutropenia and infections were seen in 52.6% and 38.5% of patients receiving BR. The median OS was not reached in both regimens ( = .25).

Conclusion: Indian patients with CLL are younger in chronological age but have higher morbidity burden. Treatment outcomes with biosimilar fixed-dose BR are comparable to those reported in the literature. Chlorambucil is still a valid option, given the economic burden of the disease and treatment.
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http://dx.doi.org/10.1200/GO.20.00032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7328099PMC
June 2020

BRAF V600E mutation detection in hairy cell leukemia-utility of archival DNA from bone marrow aspirate/imprint smear and amplification refractory mutation system.

Mol Biol Rep 2020 Jun 26;47(6):4365-4372. Epub 2020 May 26.

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

BRAF V600E is a disease defining mutation for hairy cell leukemia (HCL), which helps in its diagnosis and differentiation from morphologically similar splenic marginal zone lymphoma (SMZL) and HCL-variant (HCL-v). Forty eight cases:HCL(n = 34), SMZL(n = 11) and HCL-v(n = 3) were included. Of these, 32 were retrospective and 16 were prospective. DNA was extracted, in retrospective cases from cells obtained by smears from bone marrow aspirate/trephine imprint (BMA/BMTx) slides, and in prospective cases from peripheral blood (PB)/BMA samples. BRAF V600E mutation testing was done using ARMS-PCR. BRAF V600E mutation was positive in all HCL and negative in all SMZL and HCL-v cases. DNA extracted from BMA/BMTx slides gave results comparable to DNA extracted from PB/BMA samples. Median age of presentation for HCL (53 years) and SMZL (56 years) were quite similar, however, HCL-v (71 years) cases presented at an older age. Statistically significant differences between the three groups were seen for total leucocyte, platelet, absolute lymphocyte and monocyte counts, bone marrow-infiltration pattern, reticulin fibrosis, and an expression of CD11c, CD25, CD103, CD123, and CD200. The use of BMA/BMTx smears for DNA extraction was found to be a useful alternative to DNA extraction from formalin-fixed paraffin-embedded biopsy sections. ARMS-PCR is an efficient and specific technique to detect BRAF V600E mutation in HCL patients.
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http://dx.doi.org/10.1007/s11033-020-05509-0DOI Listing
June 2020

Paratrabecular myelofibrosis and occult mastocytosis are strong morphological clues to suspect translocation in hypereosinophilia.

Indian J Hematol Blood Transfus 2020 Apr 4;36(2):384-389. Epub 2019 Dec 4.

1Department of Hematology, Post Graduate Institute of Medical Education and Research, 5th floor, Research block A, Chandigarh, 160012 India.

To study the clinico-haematological and histopathological characteristics of rearranged hypereosinophilia/hypereosinophilic syndrome (F/P+ve HE/HES), a retrospective analysis of patients with F/P+ve HE diagnosed over a period of 43 months was performed. Peripheral blood smears, bone marrow aspirate (BMA) and biopsies (BMB) were reviewed in each case and; reticulin stain and immunohistochemistry for mast cell tryptase (MCT) and CD117 was performed. F/P+ve HE was diagnosed in a total of ten patients during study period. All patients were males with a median age of 36 years (23-44 years). The median duration of presenting complaints was 7 months (2 months-3 years) which included specific symptoms related to various organs (80% of cases). Anaemia, thrombocytopenia and splenomegaly were seen in 60%, 50% and 90% of the cases respectively. Mastocytosis was not obvious in BMA but identified by MCT on BMB in all cases. Myelofibrosis (grade ≥ 1) was seen in 80% of the cases and includes multifocal paratrabecular fibrosis in 50% of the biopsies. Our study shows that bone marrow mastocytosis and myelofibrosis are very useful morphological indicators to suspect F/P+ve HE and suggests the routine use of reticulin staining and MCT immunohistochemistry in all BMBs performed for the evaluation of HE/HES.
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http://dx.doi.org/10.1007/s12288-019-01236-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229073PMC
April 2020

Inherited Bleeding Disorders in North Indian Children: 14 years' Experience from a Tertiary Care Center.

Indian J Hematol Blood Transfus 2020 Apr 21;36(2):330-336. Epub 2019 Nov 21.

1Division of Pediatric Hemato-Oncology, Department of Pediatrics, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012 India.

Inherited bleeding disorders are not uncommon in pediatric practice: most of them being chronic, require lifelong replacement therapy. To frame a management policy, it is essential to assess the load and pattern of bleeding disorders in the local population. However, there is paucity of data reporting the clinical spectrum of coagulation and platelet function disorders in Indian children. Hence to find out the exact burden and clinico-investigational profile of these patients we conducted this study. In this retrospective case review, detailed clinical information was extracted from case records in 426 children with a suspected diagnosis of hereditary bleeding disorder registered in the Pediatric Hematology clinic of a tertiary referral centre over a period of 14 years (1998-2011) and pooled for analysis. In our cohort prevalence of hemophilia A, hemophilia B, platelet function disorders, von Willebrand disease and other rare factor deficiencies were 72%, 11%, 7%, 4% and 4% respectively. Common clinical spectrum included skin bleeds, arthropathy, mucosal bleeds. 10% had deeper tissue bleeding and 16% received replacement therapy at the first visit. Nearly 3/4th of cases were lost for follow up after the initial visit. Hemophilia A was the commonest inherited bleeding disorder in our population. Skin bleeds and arthropathy were common clinical presentations. Factor replacement therapy was restricted to a minority. There is an urgent need for establishing centres of excellence with administrative commitment for factor replacement therapy for comprehensive management of such children in resource-limited countries.
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http://dx.doi.org/10.1007/s12288-019-01233-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229128PMC
April 2020

Imatinib-Induced Hypogammaglobulinemia in Children and Adolescents with Chronic Myeloid Leukemia.

Pediatr Hematol Oncol 2020 Sep 4;37(6):539-544. Epub 2020 May 4.

Pediatric Hematology-Oncology Unit, Dept. of Pediatrics, Advanced Pediatrics Center, Chandigarh, India.

Imatinib-induced tyrosine kinase inhibition extends beyond the BCR-ABL mutation, resulting in adverse effects. We evaluated hypogammaglobulinemia as a potential 'off-target' action of imatinib in children with CML. A cross-sectional, observational study was performed. Patients with CML in chronic phase, age <18-years at diagnosis, receiving imatinib for a duration exceeding 6-months were enrolled. Serum immunoglobulin G, A, and M were measured by end-point nephelometry. Thirty patients were enrolled. The mean age at diagnosis was 10.4 ± 3.1 years (range: 5-18). The mean age at enrollment was 16.4 ± 4.1 years (range: 9-23). The median dose of imatinib was 287.5 mg/m (IQR: 267.3, 345.0). The median duration of imatinib-therapy was 6-years (IQR: 3.0, 10.3). The median (IQR) normalized levels of IgG, IgA, and IgM were 33.0% (IQR: -12.8, 58.7), 28.1% (IQR: -17.0, 90.1) and 15.9% (IQR: -9.3, 40.5), respectively. The IgG, IgA, and IgM levels were reduced in 9 (30%), 8 (27%), and 10 (33%) patients, respectively. Five (17%) patients had pan-hypogammaglobulinemia. We suggest checking immunoglobulin levels in patients with CML receiving imatinib with recurrent/unusual infections.
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http://dx.doi.org/10.1080/08880018.2020.1759739DOI Listing
September 2020

Pseudo-Rosette-Forming Blastic Plasmacytoid Dendritic Cell Neoplasm

Turk J Haematol 2020 08 15;37(3):210-211. Epub 2020 Apr 15.

Department of Hematology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

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http://dx.doi.org/10.4274/tjh.galenos.2020.2020.0141DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7463204PMC
August 2020

Transient Erythroblastopenia: An Unusual Manifestation of Macrophage Activation Syndrome in Kawasaki Disease.

J Clin Rheumatol 2020 Feb 22. Epub 2020 Feb 22.

From the Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre.

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http://dx.doi.org/10.1097/RHU.0000000000001349DOI Listing
February 2020

A Case of Myelodysplastic Syndrome in an Adult with Down Syndrome: A Rare Observation of a Well-known Pediatric Disease

Turk J Haematol 2020 05 19;37(2):137-138. Epub 2020 Feb 19.

Postgraduate Institute of Medical Education and Research, Department of Hematology, Chandigarh, India

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http://dx.doi.org/10.4274/tjh.galenos.2020.2019.0397DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7236405PMC
May 2020

Catastrophes due to missing complements: C1q deficiency lupus with Kikuchi-Fujimoto disease and macrophage activation syndrome.

Rheumatology (Oxford) 2020 07;59(7):1794

Allergy Immunology UnitDepartment of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1093/rheumatology/keaa048DOI Listing
July 2020

Genetic basis of unexplained erythrocytosis in Indian patients.

Eur J Haematol 2019 Aug 13;103(2):124-130. Epub 2019 Jun 13.

Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Objective: To evaluate the spectrum of genetic defects in Indian patients with unexplained erythrocytosis.

Methods: Fifteen families (18 patients) with unexplained erythrocytosis were enrolled after excluding polycythemia vera and secondary erythrocytosis. Focused Sanger sequencing from genomic DNA was performed for EPOR (exon 8), VHL (exons 2-3), EGLN1 (exons 2-5), EPAS1 (exon 12), and all exons of HBB, HBA1, and HBA2 genes.

Results: Eleven of the 18 patients (including two pairs of brothers) had Chuvash polycythemia, that is, homozygosity for VHL:c.598C > T (p.Arg200Trp). Three patients (two of whom were brothers) had HBB mutations associated with increased oxygen-affinity hemoglobin-one had a heterozygous Hb McKees Rocks HBB:c.438T > A (p.Tyr146*), and two brothers showed heterozygous Hb Rainier HBB:c.437A > G (p.Tyr146Cys). No pathogenic variants were found in the remaining four cases.

Conclusion: A gene-by-gene Sanger sequencing approach could determine a genetic basis for erythrocytosis in 11 of the 15 (73%) Indian families, with homozygous VHL:c.598C > T (p.Arg200Trp) being the commonest pathogenic variant. This first study from the Indian subcontinent provides a rationale for analyzing this variant in patients with suspected congenital erythrocytosis from this region. Rare first occurrences of Hb McKees Rocks and Hb Rainier in Indians are also being reported.
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http://dx.doi.org/10.1111/ejh.13267DOI Listing
August 2019

Unique characteristics of leukocyte volume, conductivity and scatter in chronic myeloid leukemia.

Biomed J 2019 04 6;42(2):93-98. Epub 2019 May 6.

Department of Internal Medicine (Adult Clinical Hematology Unit), Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Background: Modern automated hematology analyzers provide quantitative data on leukocyte size and structure that may be useful to distinguish reactive from neoplastic cellular proliferations. We compared leukocyte volume, conductivity and scatter (VCS) characteristics of chronic myeloid leukemia (CML), bcr-abl1-positive patients with those of non-neoplastic neutrophilia.

Materials And Methods: Complete blood counts and VCS data (LH750 hematology analyzers, Beckman Coulter) from 38 newly-diagnosed CML patients, 65 CML on imatinib mesylate therapy, 58 patients with elevated age-specific neutrophil counts due to varied causes, 100 pregnant women and 99 healthy controls were collated and compared. Receiver-operating-characteristic curves, logistic regression models and classification trees were studied for their abilities to distinguish various groups.

Results: Untreated CML had higher mean neutrophil volume and mean monocyte volume (MNV and MMV), mean lymphocyte scatter (MLS) and higher standard deviations of the mean neutrophil volume and conductivity (MNV-SD and MNC-SD) over all other groups (p < 0.0001 for all). MNV, MNC-SD and MLS distinguished CML from reactive neutrophilia + pregnancy groups (sensitivities 89.5%, 94.7%, 94.7% and specificities 90.6%, 95.6% and 94.0% respectively). Combination of MNV>163.0 AND MNC-SD>12.69 was 89.5% sensitive and 100% specific for CML. Two algorithmic classification-tree approaches using VCS parameters alone (i.e. without the aid of blood count parameters) correctly separated 100% cases of untreated CML from all others.

Conclusion: Successful distinction of untreated but not post-imatinib CML patients from subjects who were either normal, pregnant or had reactive neutrophilia by automated analyzer-derived cell-population data opens possibilities for their applications in diagnosing and understanding the pathogenesis of CML.
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http://dx.doi.org/10.1016/j.bj.2018.12.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6541876PMC
April 2019

Chronic Myeloid Leukemia Presenting with Fleeting Facial Nerve Palsy.

Indian J Hematol Blood Transfus 2019 Apr 29;35(2):371-372. Epub 2019 Jan 29.

1Department of Internal Medicine, Post-Graduate Institute of Medical Education and Research, Chandigarh, 160012 India.

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http://dx.doi.org/10.1007/s12288-019-01080-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439067PMC
April 2019

Adolescent and Young Adult Chronic Myeloid Leukemia in Real-World Settings: Experience from a Tertiary Care Institute in Northern India.

J Adolesc Young Adult Oncol 2019 02 1;8(1):94-97. Epub 2018 Nov 1.

2 Department of Internal Medicine (Clinical Hematology Division), PGIMER, Chandigarh, India.

The data on adolescent and young adult chronic myeloid leukemia (AYA-CML) from the Indian subcontinent are scarce. We studied characteristics of AYA-CML through a retrospective analysis of 1950 CML patients registered to a tertiary care hospital in Northern India. AYA-CML represented 22.1% of all CML patients, with cumulative overall survival (OS) of 84%, and 1 and 8 years OS of 94.2% and 74.2%, respectively. Of all cases, 8.91% patients had advanced disease at the time of diagnosis, and 13.95% had myelofibrosis in the diagnostic marrow, 79.6% had complete cytogenetic response (CCyR), and 84.5% had major molecular response (MMR). Loss of CCyR and MMR was noted in 37.2% and 28.4%, respectively. The cumulative OS was significantly better in patients on patient assistance program, and they were initiated on therapy within 3 months of symptom onset.
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http://dx.doi.org/10.1089/jayao.2018.0007DOI Listing
February 2019

Leukocyte Cell Population Data for Hematology Analyzer-Based Distinction of Clonal-versus-Non-Clonal Lymphocytosis: A Real-World Testing Experience.

Indian J Hematol Blood Transfus 2018 Oct 20;34(4):623-631. Epub 2018 Jan 20.

4Adult Clinical Hematology Unit, Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Automated blood counts revealing lymphocytosis necessitate smear reviews. Even expert morphological evaluation may however, fail to differentiate a benign-versus-malignant etiology without further testing. Automated analyser-derived quantitative data on leukocyte cell populations remain undertested for distinguishing such etiologies. Instrument manufacturers claim that if successful, they may be used to generate software flags that help under-resourced laboratories better triage hemogram specimens requiring further testing. We tested the diagnostic accuracy of volume-conductivity-scatter (VCS) indices together with complete blood count (CBC) parameters in such scenarios. We compared LH780-derived (Beckman Coulter, FL, USA) CBC + VCS parameters from patients with clonal lymphoproliferations (n = 42, including 30 chronic lymphocytic leukemia cases) versus 83 controls with absolute or relative lymphocytosis (derivation cohort). Diagnostic performances of 11 logistic regression equations derived were subsequently evaluated on two specific validation cohorts (n = 130 and n = 1465). Clonal lymphocytoses showed significantly lower hemoglobin and higher leukocyte counts but similar lymphocyte percentages (LY %) vis-à-vis controls. The most significant, albeit overlapping predictor of clonality was the absolute lymphocyte count, LY# (47.8 ± 48.4 × 10/L vs. 2.9 ± 1.4 × 10/L in clonal vs. benign cases). In eleven logistic regression equations constructed using four combinatorial approaches, only the models with LY# (highest sensitivity/specificity of 99.3%/100%) and the lymphocytic VCS parameters alone (highest sensitivity/specificity of 76.2%/90.2%) performed consistently in both validation cohorts. Lymphocytic VCS parameters were moderately successful in distinguishing benign-versus-malignant lymphocytes. Other approaches of CBC-plus-VCS parameters did not sustain their initial excellent performances in the validation cohorts, highlighting a need for careful appraisal and better standardization of automated cellular analysis technologies.
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http://dx.doi.org/10.1007/s12288-018-0921-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186258PMC
October 2018