International Journal of Hematology

International Journal of Hematology | Vol. 5, No. 11, November 2014 | pp. 81–88
DOI: 10.46882/2014/IJH/000059

Original Article

Title: Immunophenotypic differentiation of plasma cell leukemia from multiple myeloma using CD56 and CD117 expression markers

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: Plasma cell leukemia is an aggressive variant of plasma cell dyscrasia characterized by high numbers of circulating clonal plasma cells. This study investigated the immunophenotypic differences between 10 cases of primary plasma cell leukemia and 45 cases of newly diagnosed multiple myeloma using multiparameter flow cytometry panels targeting CD38, CD138, CD56, and CD117 expression levels. Clonal plasma cells were identified via strong co-expression of CD38 and CD138. CD56 expression was detected in 77.8% (35 of 45) of multiple myeloma patients but was completely absent (0.0%) in all 10 plasma cell leukemia cases (P < 0.001). Similarly, CD117 (c-kit) was expressed in 33.3% of multiple myeloma cases but was negative in the plasma cell leukemia cohort. Conversely, plasma cell leukemia cells displayed a higher density of CD45 and CD19 expression compared to standard intra-medullary myeloma counterparts. The complete absence of CD56 and CD117 coupled with CD45 upregulation marks a distinct immunophenotypic profile for plasma cell leukemia. These biomarkers highlight an altered cell adhesion mechanism that promotes extramedullary escape, assisting pathologists in diagnosing this aggressive hematological condition.

Keywords: Plasma cell leukemia, multiple myeloma, flow cytometry, CD56, CD117, immunophenotyping

Manuscript Timeline: Received: August 20, 2014; Revised: September 25, 2014; Accepted: October 14, 2014; Published: November 19, 2014

International Journal of Hematology | Vol. 5, No. 4, April 2014 | pp. 25–32
DOI: 10.46882/2014/IJH/000052

Original Article

Title: Prevalence and molecular profiles of JAK2 V617F, CALR, and MPL mutations in patients with BCR-ABL1-negative myeloproliferative neoplasms

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 diagnostic landscape of BCR-ABL1-negative myeloproliferative neoplasms has been redefined by discovering specific driver mutations. This cross-sectional study investigated the mutational prevalence and clinical correlates of JAK2 V617F, Calreticulin (CALR), and myeloproliferative leukemia virus oncogene (MPL) mutations in 72 adult patients with suspected essential thrombocythemia, polycythemia vera, or primary myelofibrosis. Genomic DNA was isolated from peripheral blood, followed by allele-specific PCR and Sanger sequencing analysis. The JAK2 V617F mutation was identified in 58.3% (42 of 72) of the entire cohort, including 92.3% of polycythemia vera and 41.9% of essential thrombocythemia cases. CALR mutations (Type 1 and Type 2) were detected in 19.4% (14 of 72) of patients, occurring exclusively in JAK2-negative essential thrombocythemia and myelofibrosis cases. MPL exon 10 mutations (W515L/K) were rare, found in only 4.2% of patients. Triple-negative status was documented in 18.1% of the cohort. Patients harboring CALR mutations displayed significantly lower white blood cell counts and a lower risk of thrombosis compared to those with JAK2 V617F mutations (P < 0.05). Screening for these driver variants optimizes diagnostic accuracy according to World Health Organization criteria.

Keywords: Myeloproliferative neoplasms, JAK2 V617F mutation, Calreticulin mutation, MPL mutation, molecular diagnostics

Manuscript Timeline: Received: January 10, 2014; Revised: February 18, 2014; Accepted: March 12, 2014; Published: April 16, 2014

International Journal of Hematology | Vol. 5, No. 3, March 2014 | pp. 17–24
DOI: 10.46882/2014/IJH/000051

Original Article

Title: Predicting early relapse in diffuse large B-cell lymphoma using serum soluble IL-2 receptor levels

Names of Authors: A. I. Ibrahim¹, C. D. Balogun², E. O. Ojo³

Authors’ Affiliations: ¹Department of Haematology, Ahmadu Bello University, Zaria, Nigeria; ²Department of Medicine and Oncology, University of Ilorin, Ilorin, Nigeria; ³Department of Chemical Pathology, Ladoke Akintola University of Technology, Ogbomoso, Nigeria

Abstract: Identifying reliable biomarkers for early relapse in diffuse large B-cell lymphoma is vital for modifying frontline treatment protocols. This prospective study evaluated the clinical utility of monitoring serum soluble interleukin-2 receptor (sIL-2R) levels in 65 newly diagnosed patients undergoing standard R-CHOP chemotherapy. Serum sIL-2R levels were quantified using an enzyme-linked immunosorbent assay at baseline, mid-treatment, and completion of therapy. Baseline sIL-2R levels were significantly higher in patients with advanced clinical stage disease (Binet/Ann Arbor III/IV) compared to early-stage cohorts (mean 2,850 ± 420 U/ml versus 840 ± 150 U/ml, P < 0.001). Following 6 treatment cycles, 73.8% (48 of 65) achieved complete remission, accompanied by a decline in sIL-2R to normal ranges (< 500 U/ml). However, 12 patients who subsequently experienced disease relapse within 12 months demonstrated a significant rebound in sIL-2R levels at a median of 2.5 months before clinical or radiological detection. High baseline and persistent mid-treatment elevation of sIL-2R correlated with poor progression-free survival (hazard ratio = 3.15, P < 0.01). Serial monitoring of serum sIL-2R serves as a sensitive, non-invasive biomarker for tracking tumor burden and predicting early relapse in lymphoma patients.

Keywords: Diffuse large B-cell lymphoma, soluble IL-2 receptor, tumor biomarker, relapse prediction, chemotherapy response

Manuscript Timeline: Received: December 12, 2013; Revised: January 20, 2014; Accepted: February 05, 2014; Published: March 14, 2014

International Journal of Hematology | Vol. 5, No. 6, June 2014 | pp. 41–48
DOI: 10.46882/2014/IJH/000054

Original Article

Title: Efficacy and compliance tracking of low-dose deferasirox therapy in transfusion-dependent beta-thalassemia major 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 red blood cell transfusions are mandatory for survival in beta-thalassemia major but lead to systemic iron overload, requiring effective chelation therapy. This 12-month prospective study evaluated the efficacy, safety, and compliance tracking of a low-dose oral deferasirox regimen (15 to 20 mg/kg/day) in 35 transfusion-dependent pediatric and young adult patients. Iron overload was monitored via serial serum ferritin levels at 3-month intervals, while monthly creatinine and liver enzyme checks tracked drug safety. Baseline mean serum ferritin was 3,450 ± 620 ng/ml. After 12 months of deferasirox therapy, mean serum ferritin fell significantly to 2,120 ± 450 ng/ml (P < 0.01) in compliant individuals. Drug adherence, assessed via pill counts and structured interviews, was high at 82.8%. Mild, transient increases in serum creatinine (> 33% above baseline) occurred in 11.4% of patients but resolved without dose interruption. Gastrointestinal side effects, mostly mild diarrhea or nausea, occurred in 22.8% of cases. Low-dose oral deferasirox demonstrates satisfactory efficacy in reducing systemic iron burden with a manageable safety profile, making it a viable options for improving compliance in low-resource environments.

Keywords: Beta-thalassemia major, iron overload, deferasirox, iron chelation therapy, ferritin

Manuscript Timeline: Received: March 15, 2014; Revised: April 24, 2014; Accepted: May 12, 2014; Published: June 18, 2014

International Journal of Hematology | Vol. 5, No. 5, May 2014 | pp. 33–40
DOI: 10.46882/2014/IJH/000053

Review Article

Title: Pathophysiology and emerging targeted therapies for the prevention of delayed hemolytic transfusion reactions in sickle cell anemia

Names of Authors: M. A. Bello¹, O. R. Eze²

Authors’ Affiliations: ¹Department of Haematology, Aminu Kano Teaching Hospital, Kano, Nigeria; ²Department of Pathology, University of Benin, Benin City, Nigeria

Abstract: Delayed hemolytic transfusion reactions represent a life-threatening immune complication in sickle cell anemia, often precipitating severe hyperhemolysis where both donor and recipient red cells undergo rapid destruction. This review explores the underlying pathophysiological mechanisms, focusing on rapid anamnestic alloantibody production against minor blood group antigens (Rh, Kell, Duffy, Kidd) and subsequent complement activation. Macrophage-mediated erythrophagocytosis, endothelial damage from free hemoglobin release, and consumption of nitric oxide contribute to a prothrombotic, hyperinflammatory state. Clinical diagnosis is challenging as it mirrors vaso-occlusive crises, causing diagnostic errors and inappropriate further transfusions. Emerging preventive strategies emphasize extensive extended red cell antigen matching and the use of prophylactic immunomodulators. Complement inhibitors like eculizumab and interleukin-6 receptor antagonists like tocilizumab represent innovative targets for cutting off the hyperhemolytic cascade. This review provides a comprehensive clinical algorithm focused on early diagnostic biomarkers, such as an unexpected drop in post-transfusion hemoglobin and elevated lactate dehydrogenase, combined with immediate immunosuppressive interventions to reduce mortality in alloyimmunized sickle cell cohorts.

Keywords: Sickle cell anemia, delayed hemolytic transfusion reaction, hyperhemolysis, alloimmunization, complement inhibitors

Manuscript Timeline: Received: February 11, 2014; Revised: March 22, 2014; Accepted: April 10, 2014; Published: May 19, 2014

International Journal of Hematology | Vol. 5, No. 2, February 2014 | pp. 9–16
DOI: 10.46882/2014/IJH/000050

Short Communication

Title: Screening for lupus anticoagulant using a simplified textrin time assay protocol in patients with recurrent pregnancy loss

Names of Authors: B. C. Akpan¹, D. E. Usman²

Authors’ Affiliations: ¹Department of Haematology, University of Calabar, Calabar, Nigeria; ²Department of Clinical Pharmacology, Ahmadu Bello University, Zaria, Nigeria

Abstract: Lupus anticoagulant is an established cause of immune-mediated obstetric complications, but standard multi-step confirmation assays can be complex for routine diagnostic laboratories. This study evaluated the screening performance of a simplified textrin time assay, which uses standard Taipan snake venom, compared to the reference diluted Russell’s viper venom time in 80 women with a history of unexplained recurrent early pregnancy loss. Solid-phase enzyme-linked immunosorbent assays also quantified anticardiolipin antibodies. Lupus anticoagulant positivity was identified in 16.2% (13 of 80) of the patient cohort using the diluted Russell’s viper venom time reference technique. The simplified textrin time protocol demonstrated a sensitivity of 92.3% (12 of 13 positive cases) and a specificity of 89.5% when evaluated against the reference method. The textrin time assay resisted interference from therapeutic low-molecular-weight heparin due to the specific direct prothrombin-activating mechanism of the venom. Utilizing the simplified snake venom clot protocol provides a cost-effective and reliable alternative for identifying lupus anticoagulant risk profiles in maternal health settings.

Keywords: Recurrent pregnancy loss, lupus anticoagulant, textrin time, antiphospholipid syndrome, thrombophilia

Manuscript Timeline: Received: November 15, 2013; Revised: December 22, 2013; Accepted: January 11, 2014; Published: February 18, 2014