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Gurjeewan Garewal

Publications and source records attributed to Gurjeewan Garewal.

16 recordsLinked to original sources
PubMed2006

The utility of bone marrow examination in renal transplantation: nine years of experience from north India.

Renal transplantation may be complicated by persistent fever and cytopenia. Bone marrow examination, though painful and invasive, may be of value because it can be performed in most peripheral hospitals, and the results are rapidly available. In a retrospective analysis, clinical records, marrow aspirates, and trephine biopsies were assessed to determine the indications for bone marrow examination and the findings in 132 patients over a 9-year period. In 7 of 61 (11%) patients with fever, a specific infection or a malignancy was identified, and hypocellularity was seen in 85% of cases. Nutritional deficiency with megaloblastosis or diminished iron stores was noted in 29% of cases. Malignancies were relatively infrequent (3%). Overall, in 12% of the cases, bone marrow examination provided useful information. Examination of the bone marrow may be a useful diagnostic test in pyrexia of unknown origin and cytopenia, particularly in developing countries.

Biopsy↗
PubMed2006

Automated reticulocyte response is a good predictor of bone-marrow recovery in pediatric malignancies.

The authors attempted to establish the benefits of flow cytometry-based reticulocyte analysis over absolute neutrophil count (ANC) recovery. Serial hemograms of 18 pediatric cases of hematologic malignancies were analyzed until day 35 of chemotherapy. Immature reticulocyte fraction (IRF) showed early recovery in 44.4% of cases compared to ANC. Since reticulocyte fractions are not influenced by infections, they are a better parameter of bone marrow regeneration than ANC. The study shows that IRF can act as a harbinger of regenerating bone marrow activity in patients with persistent neutropenia and guide the modulation of antibiotic strategies in these patients.

Adult↗
PubMed2005

Nonalcoholic steatohepatitis in Asian Indians is neither associated with iron overload nor with HFE gene mutations.

AIM: The pathogenesis of occurrence of liver inflammation and fibrosis in patients with nonalcoholic steatohepatitis (NASH) is not completely understood. Other than insulin resistance, iron abnormalities have been thought to be one of the triggering factors. Therefore, our aim was to study the role of iron abnormalities and HFE gene mutations in patients with NASH. METHODS: Thirty-one patients of NASH diagnosed on the basis of clinical examination biochemistry, ultrasonography and liver biopsy (n = 14) were included in the study. Serum iron parameters (n = 23) (iron, ferritin, total iron-binding capacity and transferrin saturation), Perls' iron staining on liver biopsies (n = 14) and HFE gene mutations (C282Y and H63D) (n = 16) were studied in these patients. The association between iron staining, necroinflammatory activity and fibrosis stage on liver biopsies was also determined. RESULTS: Elevated serum iron, ferritin and transferrin saturation above 55% were observed in 4.3% of patients. On histology, 71% of the patients had negative iron staining, 21.4% had 1+ staining, 7.2% had 2+ staining and none had 3+ or 4+ staining. There was no association between the degree of iron staining and necroinflammatory activity (P = 0.55) and fibrosis stage (P = 0.09) on histology. None of the patients had C282Y HFE gene mutation and four patients (25%) were found to be heterozygotes for H63D gene mutation. CONCLUSION: Our study does not favor iron overload and HFE gene mutations as major factors in the pathogenesis of NASH in Asian Indians.

Adult↗
PubMed2005

Nucleotide -88 (C-T) promoter mutation is a common beta-thalassemia mutation in the Jat Sikhs of Punjab, India.

A study of beta-gene mutations in Jat Sikhs, a subcaste of Punjabis, revealed a very high prevalence (46%: 41/88) of the mild beta++ promoter region mutation -88 (C-T). Sixteen individuals presenting in homozygous form were clinically mild. Un-transfused patients had characteristic hematological findings: high Hb F (38.1-68.6%, mean 47.4%), high Hb A2 (5.7-9.8%, mean 6.88%), and the rest had adult hemoglobin. The 19 subjects with compound heterozygosity for -88 (C-T) and another beta-gene mutation presented both as thalassemia intermedia (four cases) and as thalassemia major (15 cases). One of the four patients with the milder phenotype had a second mild mutation, CAP+1 (beta++). In the other three cases with the milder phenotype, the second mutation was associated with the presence of the XmnI Ggamma polymorphism. Notably, the XmnI Ggamma was negative in all the -88 (C-T) alleles. None of the patients had associated alpha-thalassemia even in the thalassemia intermedia group. Haplotype analysis of the -88 (C-T) homozygous cases showed a single haplotype (+ - - - - + -) in all but two individuals. This haplotype is distinct from those described in the Africans with homozygous -88 (C-T), suggesting that the mutation in our population occurred independently.

Adolescent↗
PubMed2005

Interferon therapy in congenital dyserythropoietic anemia type I/II.

Congenital dyserythropoietic anemia (CDA) is a rare disorder, characterized by the association of ineffective erythropoiesis, variable degree of anemia, and erythroblastic morphological abnormalities. alpha-Interferon has been reported to be effective in type I CDA, but efficacy in other types of CDA is uncertain. Encouraged by the reports, we evaluated the efficacy of alpha-interferon in 6 children with CDA. Diagnosis of CDA was established on the basis of clinical profile, distinct morphological findings on light microscopy, and the Ham's test, following the exclusion of the more common causes of hemolysis. Erythrocyte agglutinability and lysis to anti-i and anti-l sera, electron microscopy, and SDS-polyacrylamide gel electrophoresis were not performed, due to nonavailability. There were 3 cases, each, with type I and type II CDA. The mean age was 5.5 years (range: 6 months to 11.5 years). Five of the 6 patients were transfusion dependent. alpha-Interferon was administered subcutaneously for a mean duration of 19 weeks (range: 12-30). The dose ranged from 2.6 to 6.5 million IU/m2/dose. The frequency of injections varied from thrice weekly to alternate days. No favorable effect on hemoglobin, reticulocyte count, or transfusion frequency was observed. alpha-Interferon therapy was found to be ineffective in all the patients. These observations question the use of interferon in CDA until further studies in a larger number of patients establish its efficacy.

Anemia, Dyserythropoietic, Congenital↗
PubMed2005

Hematological disorders in Down syndrome: ten-year experience at a Tertiary Care Centre in North India.

A total of 239 cases of Down syndrome (DS) were seen in the genetic clinic between 1992 and 2003, of which of 15 had hematological manifestations at presentation. These comprised 4 cases of transient myeloproliferative disorder (TMD), 3 cases of TMD/acute leukemia, 4 cases of acute leukemia (AL), 2 of dual deficiency anemia, and 1 case each of myelofibrosis and idiopathic thrombocytopenia. This study emphasizes the fact that an abnormal hemogram in a DS patient does not necessarily indicate AL/TMD, as a considerable number of the cases in this study had other hematological abnormalities. TMD can be differentiated from acute leukemia only on follow-up.

Case-Control Studies↗
PubMed2005

Investigation of persistent hypochromic microcytosis unmasks hemoglobin Evanston [alpha 14 (A12) Try--> Arg] in a patient of cyclic thrombocytopenia preceding Takayasu's disease.

We report an unusual north Indian patient with Hemoglobin Evanston [alpha 14 (A12) Try --> Arg] who had acquired cyclic thrombocytopenia (10-1230 x 10(9)/l periodic oscillation of four week duration) which recovered without any specific therapy. She later developed Takayasu's disease and underwent three corrective stents. She is presently in clinical remission and is on regular follow up. To the best of our knowledge our patient is the first report of Hb Evanston from the indigenous population of India and highlights the need to look for point mutations in the alpha globin gene, which may interact with thalassemia or other hemoglobinopathies, in atypical cases. The association of these three disorders in our patient is possibly unrelated though an immune basis for the cyclic thrombocytopenia and Takayasu's disease is likely as seen in this report.

Adult↗
PubMed2005

Prevalence of the H63D mutation of the HFE in north India: its presence does not cause iron overload in beta thalassemia trait.

OBJECTIVES: To determine the allele frequency in the north Indian population of the two mutations in the HFE gene, the C282Y and H63D, which are responsible for causing hereditary haemochromatosis particularly in Caucasians of north European descent. We also wanted to correlate these mutations with the iron status in beta thalassemia traits. PATIENTS AND METHODS: Sixty normal subjects and 215 individuals with beta thalassemia trait from north India were screened for the C282Y and H63D by polymerase chain reaction-restriction fragment-length polymorphism (PCR-RFLP). We studied the iron status in these subjects and correlated the same with the HFE gene mutations. RESULTS: On screening for the C282Y gene mutation, all individuals were detected to be of the wild-type. The overall allele frequency of H63D was 9.09% with three individuals being homozygous for 63D. No statistically significant difference in the iron status was detected between the individuals of the wild-type and mutant for H63D. Haplotyping of the homozygous 63D alleles revealed the pattern to be identical to the Europeans. CONCLUSIONS: Our study shows that H63D is prevalent and C282Y is rare in north Indians and the presence of 63D mutation does not increase body iron as measured by serum ferritin in beta thalassemia traits. Haplotype of H63D gene mutation is of an European haplotype, indicating a common origin.

Alleles↗
PubMed2005

Comparative study of myelodysplastic syndromes and normal bone marrow biopsies with conventional staining and immunocytochemistry.

OBJECTIVE: To study the histomorphometric features of megakaryocytic elements in bone marrow trephine biopsies of various subtypes of myelodysplastic syndrome (MDS). STUDY DESIGN: Comparative image morphometry using hematoxylin-eosin-stained and CD 61-stained trephine biopsies was carried out on 40 cases of MDS and 10 normal subjects to analyze the megakaryocytes objectively. The various variables analyzed were number of megakaryocytes and micromegakaryocytes, area, perimeter and diameter of the megakaryocytic elements. RESULTS: The mean number of megakaryocytes was lower in cases of MDS as compared to the normals in all except for a single case of hypoplastic MDS, in which the megakaryocytes were more abundant (3.6 per high-power field [hpf]). No micromegakaryocytes were observed in the 2 cases of chronic myelomonocytic leukemia. The mean area, perimeter and diameter of megakaryocytes decreased significantly on immunostaining with CD 61. CONCLUSION: The mean number of megakaryocytes per hpf was lower in the cases of MDS as compared to normal cases on hematoxylin-eosin. However, on CD 61 staining the number of megakaryocytes per hpf increased in cases of MDS. Micromegakaryocytes were seen in scanty numbers in the normals but increased in MDS cases and increased significantly on CD 61 immunostaining. The mean area, perimeter and diameter of megakaryocytes decreased significantly on immunostaining with CD 61, indicating the increased numbers of micromegakaryocytes in MDS. Hence, immunostaining is an efficient method of detecting increased numbers of megakaryocytes and micromegakaryocytes that would ordinarily be missed by using routine hematoxylin-eosin staining.

Anemia, Aplastic↗
PubMed2004

Antiphospholipid antibodies in children with systemic lupus erythematosus: a prospective study in northern India.

Twenty-seven children with systemic lupus erythematosus (SLE) were tested for antiphospholipid antibodies (APLA), i.e. lupus anticoagulant and immunoglobulin (Ig)G or IgM anticardiolipin antibodies (ACLA), with beta-2 glycoprotein I as cofactor, in a single-centre, prospective study over 2 years. Eighteen patients (67%) tested positively for one or the other APLA during the course of the study. Twelve children (44%) tested positively for IgG ACLA and ten (37%) for IgM ACLA, whereas eight (30%) were positive for lupus anticoagulant. In two patients with thrombosis, IgG anticardiolipin positivity was seen to be variable. Unlike the results of most other reports in the literature, lupus anticoagulant positivity was not consistently associated with thrombosis. A majority of the children (83%) tested positively for ACLA during disease activity. Immunoglobulin G and IgM ACLA positivity did not correlate significantly with disease status. The results of this prospective study would indicate that, though frequently present, APLA may be unable to be predictive of disease behaviour in children with SLE.

Adolescent↗
PubMed2004

Image morphometry of acute leukemias. Comparison between lymphoid and myeloid subtypes.

OBJECTIVE: To determine if image morphometry has any role in distinguishing blasts of acute lymphoblastic leukemia (ALL-L2) from those of acute myeloid leukemia (AML-M1) and (AML-M2). STUDY DESIGN: Ten cases each of ALL-L2, AML-M1 and AML-M2 diagnosed according to the French-American-British criteria were studied. In all cases May-Grünwald-Giemsa-stained bone marrow aspiration smears were obtained. At least 100 blast cells from each case were subjected to analysis randomly with an image cytometer using Leica Quantimet 600 software (Cambridge, U.K.). The area, convex area, length, width, perimeter, convex perimeter, roundness, total optical density, average optical density and pixel grey value variance of the nuclei were measured by random selection of cells using a 40:1 objective (1 pixel = 0.446 micron). RESULTS: Mann Whitney's nonparametric test showed that there was considerable overlap of morphometric variables between the 3 subtypes. Though statistical significance was found in "roundness" between blasts of AML-M1 and ALL-L2, power analyses (sample size of 100 blasts of each subtype) did not show sufficient power for this variable. However, between blasts of ALL-L2 and AML-M2, "average optical density" and "pixel grey value variance" were statistically significant with full power using power analyses. CONCLUSION: Image morphometry may be helpful in differentiating blasts from lymphoid and myeloid leukemic subtypes.

Biopsy, Needle↗
PubMed2003

Platelet function disorders.

Platelet function disorders are a rare cause of bleeding in hematological practice. The inherited variety includes defects in platelet adhesion, aggregation, secretion and platelet procoagulant activities. The clinical presentation is usually mild with mainly mucocutaneous bleeds. Milder phenotypes may pass off unrecognized in childhood and present later in life. Testing includes a coagulation screen, which may show a prolonged bleeding time, followed by platelet aggregation tests with agonists. Flow cytometry for platelet surface markers, membrane glycoprotein analysis and facilities for identification of the genetic defect are usually available in research laboratories. Recent advances have opened vistas for antenatal testing. Bone marrow transplantation is the only curative therapeutic option available.

Blood Platelet Disorders↗
PubMed2003

Congenital dyserythropoietic anemia: clinical and hematological profile.

Congenital dyserythropoietic anemia (CDA) is a rare disorder, which manifests clinically with varying degrees of anemia and hepatosplenomegaly. These features are not pathognomic and a diagnosis of CDA is generally considered after other causes of chronic hemolytic anemia have been ruled out. The clinico-hematological profile of 10 patients with CDA is presented in this communication. Six patients had CDA type II and four had CDA type I. Age at onset of pallor ranged from birth to 9 years. Blood transfusion requirements varied from nil to monthly. This is the first report of CDA type I from India.

Anemia, Dyserythropoietic, Congenital↗