International Journal of Hematology

International Journal of Hematology | Vol. 6, No. 12, December 2015 | pp. 89–96
DOI: 10.46882/2015/IJH/000072

Original Article

Title: Prevalence and clinical correlates of JAK2 V617F allele burden variations in primary myelofibrosis

Names of Authors: G. M. Babalola¹, I. N. Nwosu², K. S. Abubakar³

Authors’ Affiliations: ¹Department of Haematology and Blood Transfusion, Lagos University Teaching Hospital, Lagos, Nigeria; ²Department of Medicine, University of Nigeria, Nsukka, Nigeria; ³Department of Pathology, Bayero University, Kano, Nigeria

Abstract: The specific mutant allele burden of the JAK2 V617F mutation may influence the phenotypic expression and clinical severity of myeloproliferative neoplasms. This study cross-sectionally evaluated the relationship between quantitative JAK2 V617F allele burdens and baseline disease complications in 40 adult patients diagnosed with primary myelofibrosis. Genomic DNA was isolated from peripheral blood samples, and allele burdens were quantified utilizing real-time polymerase chain reaction assays. The overall prevalence of the JAK2 V617F mutation within this cohort was 62.5% (25 of 40). The median allele burden among positive cases was 42.4%. Patients demonstrating a high allele burden (≥ 50.0%) exhibited significantly higher baseline white blood cell counts and an increased prevalence of massive splenomegaly (> 10 cm below the costal margin) compared to low allele burden variants (P < 0.05). Conversely, a high allele burden correlated with lower overall survival metrics due to accelerated leukemic transformation. Quantifying driver mutation percentages provides valuable prognostic insights, helping clinicians identify primary myelofibrosis patients at high risk for thromboembolic and fibrotic progression.

Keywords: Primary myelofibrosis, JAK2 V617F mutation, allele burden, splenomegaly, molecular prognosis

Manuscript Timeline: Received: September 10, 2015; Revised: October 18, 2015; Accepted: November 05, 2015; Published: December 14, 2015

International Journal of Hematology | Vol. 7, No. 4, April 2016 | pp. 25–32
DOI: 10.46882/2016/IJH/000076

Original Article

Title: Evaluation of automated nucleated red blood cell enumeration parameters in predicting bone marrow stress in acute leukemias

Names of Authors: E. F. Chinedu¹, G. H. Haruna², I. J. Balogun³

Authors’ Affiliations: ¹Department of Haematology, University of Nigeria Teaching Hospital, Enugu, Nigeria; ²Department of Medicine, Lagos State University Teaching Hospital, Ikeja, Nigeria; ³Department of Chemical Pathology, University of Ilorin, Ilorin, Nigeria

Abstract: The presence of nucleated red blood cells (NRBCs) in adult peripheral blood reflects disruption of the bone marrow-blood barrier, frequently indicating severe bone marrow infiltration. This study evaluated the performance and clinical significance of automated nucleated red blood cell counts, derived from a fluorescent flow cytometry cell counter, for predicting tumor burden in 48 adult patients with newly diagnosed acute myeloid or lymphoblastic leukemia. Automated parameters were validated via manual peripheral blood film examinations. Circulating nucleated red blood cells were detected in 64.5% (31 of 48) of leukemia patients, with a mean concentration of 4.8 ± 1.2 NRBC per 100 white blood cells. High absolute nucleated red blood cell counts correlated positively with bone marrow blast percentages (r = 0.56, P < 0.01) and inversely with total hemoglobin values. Furthermore, persistent elevations of nucleated red blood cells following initial induction therapy predicted primary chemoresistance or early disease relapse. Utilizing automated erythroblast enumeration provides a rapid, cost-effective indicator of severe marrow infiltration and systemic bone marrow stress.

Keywords: Nucleated red blood cells, acute leukemia, tumor burden, cell counter flags, marrow infiltration

Manuscript Timeline: Received: January 12, 2016; Revised: February 20, 2016; Accepted: March 08, 2016; Published: April 14, 2016

International Journal of Hematology | Vol. 6, No. 4, April 2015 | pp. 25–32
DOI: 10.46882/2015/IJH/000064

Original Article

Title: Association between serum soluble transferrin receptor levels and macrovascular complications in adult sickle cell anemia patients

Names of Authors: S. T. Adeyemi¹, U. V. Okoye², W. X. Salami³

Authors’ Affiliations: ¹Department of Haematology and Blood Transfusion, Obafemi Awolowo University, Ile-Ife, Nigeria; ²Department of Medicine, University of Nigeria Teaching Hospital, Enugu, Nigeria; ³Department of Paediatrics, Ahmadu Bello University, Zaria, Nigeria

Abstract: Chronic hemolysis in sickle cell anemia induces compensatory erythroid hyperplasia and endothelial damage, increasing the risk of macrovascular events. This study cross-sectionally evaluated the relationship between serum soluble transferrin receptor (sTfR) levels, a biomarker for erythroid marrow activity, and macrovascular complications, including ischemic stroke and pulmonary hypertension indices, in 92 adult patients with stable sickle cell anemia (HbSS). Serum sTfR was measured using an enzyme-linked immunosorbent assay, and pulmonary hypertension risk was estimated via tricuspid regurgitant jet velocity on Doppler echocardiography. Patients with elevated sTfR levels (≥ 8.5 μg/ml) showed a significantly higher mean tricuspid regurgitant jet velocity (2.85 ± 0.35 m/s versus 2.15 ± 0.20 m/s in lower sTfR cohorts, P < 0.01). A history of stroke correlated positively with high sTfR concentrations (odds ratio = 3.24, P < 0.05). Furthermore, sTfR levels correlated inversely with total hemoglobin values (r = -0.54, P < 0.001) and positively with serum lactate dehydrogenase. Elevated soluble transferrin receptor concentrations reflect intense erythroid drive linked to chronic hemolysis, serving as a useful biomarker for identifying patients vulnerable to severe hemolytic endothelial phenotypes and macrovascular injury.

Keywords: Sickle cell anemia, soluble transferrin receptor, chronic hemolysis, pulmonary hypertension, stroke

Manuscript Timeline: Received: January 08, 2015; Revised: February 18, 2015; Accepted: March 08, 2015; Published: April 14, 2015

International Journal of Hematology | Vol. 6, No. 7, July 2015 | pp. 49–56
DOI: 10.46882/2015/IJH/000067

Case Report

Title: Extramedullary relapse of acute promyelocytic leukemia in the central nervous system following complete hematological remission

Names of Authors: K. L. Musa¹, M. N. Lawal², O. P. Dikko³

Authors’ Affiliations: ¹Department of Haematology, Federal Medical Centre, Katsina, Nigeria; ²Department of Oncology, Bayero University, Kano, Nigeria; ³Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria

Abstract: Extramedullary relapse of acute promyelocytic leukemia is a rare complication following successful differentiation therapy using all-trans retinoic acid. We report a 28-year-old male who achieved sustained complete cytogenetic and hematological remission following induction and consolidation for acute promyelocytic leukemia harboring the t(15;17) PML-RARA rearrangement. Fourteen months post-consolidation, he presented with progressive severe headache, vomiting, and bilateral papilledema, without systemic symptoms. Full blood counts and bone marrow biopsy confirmed persistent marrow remission with normal hematopoietic lineages. However, lumbar puncture revealed elevated opening pressure and a leukocyte count of 140/μl. Cytocentrifuge examination of cerebrospinal fluid demonstrated numerous promyeloblasts with Auer rods. Molecular real-time PCR on cerebrospinal fluid confirmed the presence of the PML-RARA transcript, establishing a central nervous system relapse. Intrathecal chemotherapy using cytarabine and methotrexate was initiated immediately, combined with systemic arsenic trioxide. Complete clearance of blastic clones from the cerebrospinal fluid was achieved after 6 weekly cycles. This case demonstrates that the central nervous system can serve as a pharmacological sanctuary for promyelocytic clones, highlighting the need to investigate neurological symptoms even during complete marrow remission.

Keywords: Acute promyelocytic leukemia, extramedullary relapse, central nervous system, all-trans retinoic acid, cerebrospinal fluid

Manuscript Timeline: Received: April 18, 2015; Revised: May 22, 2015; Accepted: June 10, 2015; Published: July 16, 2015

International Journal of Hematology | Vol. 6, No. 8, August 2015 | pp. 57–64
DOI: 10.46882/2015/IJH/000068

Original Article

Title: Evaluation of automated immature reticulocyte fraction parameters in distinguishing vitamin B12 deficiency from bone marrow aplasia

Names of Authors: Q. S. Abubakar¹, U. T. Maina²

Authors’ Affiliations: ¹Department of Haematology, National Hospital, Abuja, Nigeria; ²Department of Pathology, Bayero University, Kano, Nigeria

Abstract: Pancytopenia with macrocytic indices requires differentiation between nutritional megaloblastic deficiencies and primary bone marrow failure syndromes. This study evaluated the diagnostic performance of the automated immature reticulocyte fraction (IRF) in separating vitamin B12 deficiency from aplastic anemia in 60 patients presenting with unexplained pancytopenia. Full blood counts and reticulocyte maturity indices were measured using a fluorescent flow cytometry hematology analyzer, and diagnoses were confirmed via bone marrow biopsy and serum B12 assays. Vitamin B12 deficiency was diagnosed in 35 patients, while 25 were confirmed as aplastic anemia. The mean immature reticulocyte fraction was significantly higher in vitamin B12 deficient patients compared to the aplastic anemia cohort (0.34 ± 0.08 versus 0.06 ± 0.02, P < 0.001), reflecting an active but ineffective erythroid drive in megaloblastosis versus a total lack of bone marrow activity in aplasia. Receiver operating characteristic analysis established an immature reticulocyte fraction threshold above 0.18 as optimal for identifying megaloblastic processes, achieving a sensitivity of 88.5% and a specificity of 84.0%. Measuring the immature reticulocyte fraction provides a rapid, automated tool that helps differentiate causes of marrow failure before invasive biopsy results are available.

Keywords: Immature reticulocyte fraction, pancytopenia, vitamin B12 deficiency, aplastic anemia, erythropoiesis

Manuscript Timeline: Received: May 12, 2015; Revised: June 20, 2015; Accepted: July 09, 2015; Published: August 14, 2015

International Journal of Hematology | Vol. 6, No. 9, September 2015 | pp. 65–72
DOI: 10.46882/2015/IJH/000069

Original Article

Title: Immunophenotypic profile and clinical staging correlates of multiple myeloma variants using a concise flow cytometry panel

Names of Authors: W. A. Adebayo¹, X. Y. Emeka², Z. Z. Salami³

Authors’ Affiliations: ¹Department of Haematology, University of Ibadan, Ibadan, Nigeria; ²Department of Haematology, University of Calabar Teaching Hospital, Calabar, Nigeria; ³Department of Pathology, Lagos University Teaching Hospital, Lagos, Nigeria

Abstract: Multiparameter flow cytometry immunophenotyping plays an important role in identifying aberrant plasma cells and establishing risk architecture in multiple myeloma. This prospective study characterized surface antigen expressions using a concise panel (CD19, CD38, CD45, CD56, CD138) in 52 newly diagnosed multiple myeloma patients and evaluated correlations with the International Staging System (ISS). Clonal plasma cells were identified via bright CD38 and CD138 co-expression. Aberrant CD56 expression was detected in 71.1% (37 of 52) of the cohort, while 88.4% demonstrated a complete absence of CD19 and CD45 markers. Patients presenting with an isolated CD56-negative/CD45-positive immunophenotype (15.3% of cases) displayed significantly higher baseline serum beta-2 microglobulin levels and were categorized predominantly in ISS Stage III (P < 0.01). Furthermore, this subgroup showed an elevated prevalence of extramedullary disease features. Utilizing a concise five-marker flow cytometry protocol provides rapid confirmation of clonal plasma cell anomalies, with specific antigen loss or retention offering helpful diagnostic indicators for aggressive multiple myeloma phenotypes.

Keywords: Multiple myeloma, flow cytometry, CD56, CD138, immunophenotyping, clinical staging

Manuscript Timeline: Received: June 15, 2015; Revised: July 24, 2015; Accepted: August 12, 2015; Published: September 15, 2015