International Journal of Hematology

International Journal of Hematology | Vol. 9, No. 11, November 2018 | pp. 81–88
DOI: 10.46882/2018/IJH/000107

Case Report

Title: Acquired Factor X inhibitor development presenting with spontaneous massive hemarthrosis in an elderly patient: Successful eradication with oral cyclosporine

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

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

Abstract: Spontaneous development of neutralizing autoantibodies against coagulation Factor X is an exceptionally rare clinical condition that causes catastrophic bleeding complications in elderly individuals. We report a 74-year-old male who presented with sudden, unprovoked, and massive swelling of his right knee joint accompanied by hypovolemic shock. Arthrocentesis yielded 450 ml of gross blood, confirming acute hemarthrosis without prior trauma or anticoagulant therapy. Coagulation profiles demonstrated concurrent prolongation of both prothrombin time (48.5 seconds) and activated partial thromboplastin time (88.4 seconds). A 1:1 mixing study with normal pooled plasma failed to correct either parameter, pointing to a common pathway inhibitor. Functional factor assays confirmed severely depressed Factor X activity (< 1.5%), and a Bethesda assay quantified a Factor X inhibitor titer of 22.0 Bethesda Units. Hemostasis was achieved using prothrombin complex concentrates (50 IU/kg every 12 hours) alongside supportive measures. Immunosuppressive therapy with high-dose corticosteroids was unsuccessful. Subsequently, oral cyclosporine-A (3 mg/kg/day) was initiated, successfully clearing the inhibitor (0 BU) and normalizing Factor X levels (88.4%) by week 6. This case highlights the need to consider common pathway inhibitors in elderly patients with spontaneous hemarthrosis.

Keywords: Acquired factor X inhibitor, hemarthrosis, common pathway, prothrombin complex concentrate, cyclosporine

Manuscript Timeline: Received: August 18, 2018; Revised: September 24, 2018; Accepted: October 10, 2018; Published: November 15, 2018

International Journal of Hematology | Vol. 9, No. 5, May 2018 | pp. 33–40
DOI: 10.46882/2018/IJH/000101

Original Article

Title: Serum cytokine kinetics and bone marrow apoptotic indices in patients with severe aplastic anemia

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, University of Ilorin, Ilorin, Nigeria; ³Department of Chemical Pathology, Ladoke Akintola University of Technology, Ogbomoso, Nigeria

Abstract: Immune-mediated bone marrow failure is heavily driven by T-cell-induced apoptosis of hematopoietic progenitor lines. This prospective study evaluated serum tumor necrosis factor-alpha (TNF-alpha) and interferon-gamma (IFN-gamma) kinetics alongside bone marrow CD34+ cell apoptotic indices in 38 newly diagnosed adult patients with severe aplastic anemia. Serum cytokine levels were quantified using an enzyme-linked immunosorbent assay, while flow cytometry determined the annexin-V positivity rates on harvested bone marrow CD34+ stem cells. Patients with severe aplastic anemia exhibited significantly elevated baseline serum IFN-gamma (42.6 ± 8.4 pg/ml) and TNF-alpha (68.4 ± 12.2 pg/ml) concentrations compared to healthy controls (P < 0.001). Apoptotic markers on CD34+ precursors were markedly elevated, with a mean index of 48.5 ± 6.4% versus 4.2 ± 1.1% in control pools. High baseline cytokine levels correlated inversely with absolute neutrophil counts (r = -0.54, P < 0.01). Following 12 weeks of immunosuppressive therapy with cyclosporine-A, responsive patients demonstrated a significant reduction in serum IFN-gamma levels corresponding to a recovery in peripheral blood counts. Tracking serum cytokine kinetics offers an accessible, non-invasive diagnostic monitor for evaluating microenvironmental immune stress and therapeutic responses in bone marrow failure syndromes.

Keywords: Aplastic anemia, interferon-gamma, tumor necrosis factor-alpha, apoptosis, bone marrow failure

Manuscript Timeline: Received: February 10, 2018; Revised: March 18, 2018; Accepted: April 05, 2018; Published: May 14, 2018

International Journal of Hematology | Vol. 8, No. 7, July 2017 | pp. 49–56
DOI: 10.46882/2017/IJH/000091

Original Article

Title: Evaluation of absolute immature reticulocyte fraction and plasma erythropoietin variants in response to recombinant human erythropoietin in aplastic anemia

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, University of Ilorin, Ilorin, Nigeria; ³Department of Chemical Pathology, Ladoke Akintola University of Technology, Ogbomoso, Nigeria

Abstract: Recombinant human erythropoietin therapy may benefit a subset of aplastic anemia patients, but pre-treatment kinetic predictors remain poorly defined. This prospective clinical study evaluated baseline plasma endogenous erythropoietin concentrations and absolute immature reticulocyte fraction (IRF) variations in 35 patients with moderate aplastic anemia receiving high-dose recombinant human erythropoietin therapy (10,000 IU subcutaneously three times weekly) over a 24-week period. Full blood counts were monitored bi-weekly, and clinical response was defined as transfusion independence or a stable hemoglobin increase exceeding 2.0 g/dl. Erythroid response was achieved in 34.2% (12 of 35) of the patients. Responders demonstrated a significantly lower baseline endogenous erythropoietin level (mean 142.5 ± 24.5 mIU/ml) compared to non-responders (mean 850.4 ± 112.0 mIU/ml, P < 0.001), reflecting a less saturated bone marrow feedback loop. Following 4 weeks of intervention, responsive patients displayed a distinct spike in the immature reticulocyte fraction from a baseline of 0.04 ± 0.01 to 0.18 ± 0.03 (P < 0.01). Quantifying baseline endogenous erythropoietin coupled with early immature reticulocyte tracking provides an affordable, predictive diagnostic framework for identifying aplastic anemia cohorts likely to benefit from growth factor regimens.

Keywords: Aplastic anemia, recombinant erythropoietin, immature reticulocyte fraction, bone marrow failure, erythropoiesis

Manuscript Timeline: Received: April 14, 2017; Revised: May 20, 2017; Accepted: June 08, 2017; Published: July 15, 2017

International Journal of Hematology | Vol. 8, No. 5, May 2017 | pp. 33–40
DOI: 10.46882/2017/IJH/000089

Original Article

Title: Immunophenotypic profile and differentiation of mantle cell lymphoma from chronic lymphocytic leukemia 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: Mantle cell lymphoma frequently presents with peripheral blood and bone marrow involvement, clinically mimicking chronic lymphocytic leukemia due to shared CD5 expression. This prospective study characterized surface antigen expressions using a concise multi-color flow cytometry panel (CD5, CD19, CD20, CD23, and FMC7) in 48 adult patients presenting with persistent absolute lymphocytosis. Mantle cell lymphoma was confirmed histologically and via t(11;14) CCND1 translocation screens in 14 cases, while 34 were diagnosed with chronic lymphocytic leukemia. Flow cytometric analysis revealed that all 14 mantle cell lymphoma patients demonstrated strong surface CD20 expression, bright FMC7 expression, and a complete absence of CD23 markers. In contrast, chronic lymphocytic leukemia cases showed uniform CD23 positivity and dim CD20 expression (P < 0.001). A Matutes immunophenotypic score of 1 or 2 was highly characteristic of mantle cell lymphoma, separating it from leukemia variants (score ≥ 4). Utilizing this concise five-marker flow cytometry protocol provides rapid and reliable lineage separation, helping pathologists identify aggressive mantle cell expansions in low-resource environments.

Keywords: Mantle cell lymphoma, chronic lymphocytic leukemia, flow cytometry, FMC7, CD23, immunophenotyping

Manuscript Timeline: Received: February 18, 2017; Revised: March 24, 2017; Accepted: April 10, 2017; Published: May 17, 2017

International Journal of Hematology | Vol. 8, No. 2, February 2017 | pp. 9–16
DOI: 10.46882/2017/IJH/000086

Original Article

Title: Evaluation of baseline plasma D-dimer levels as a prognostic predictor in patients presenting with multiple myeloma

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: Plasma cell dyscrasias induce a profound hypercoagulable state due to tumor-associated cytokine cascades and endothelial activation, yet the clinical utility of D-dimer as an independent survival predictor remains under-studied. This prospective study evaluated baseline plasma D-dimer concentrations in 54 newly diagnosed adult patients with multiple myeloma to track correlations with the International Staging System (ISS) and 1-year progression-free survival outcomes. Plasma D-dimer was quantified via immunoturbidimetric assays before initiating standard bortezomib-based induction chemotherapy. Elevated baseline D-dimer (> 1.50 mg/L) was detected in 29.6% (16 of 54) of the myeloma patients. Multivariable Cox proportional hazards regression analysis identified an elevated baseline D-dimer concentration as an independent predictor of premature mortality and early disease progression (hazard ratio = 3.42, P < 0.01). Furthermore, high D-dimer levels correlated with advanced tumor stages (ISS Stage III) and an increased prevalence of acute deep vein thrombosis. Pre-treatment plasma D-dimer screening provides valuable prognostic utility, helping clinicians identify multiple myeloma patients at high risk for hypercoagulable complications and aggressive clonal behavior.

Keywords: Multiple myeloma, D-dimer, hypercoagulability, progression-free survival, tumor biomarker

Manuscript Timeline: Received: November 11, 2016; Revised: December 20, 2016; Accepted: January 08, 2017; Published: February 16, 2017

International Journal of Hematology | Vol. 8, No. 3, March 2017 | pp. 17–24
DOI: 10.46882/2017/IJH/000087

Case Report

Title: Delayed hemolytic transfusion reaction presenting with severe priapism and hyperhemolysis in an adult patient with sickle cell anemia

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

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

Abstract: Delayed hemolytic transfusion reactions represent underdiagnosed, life-threatening immune events in sickle cell anemia that can manifest with severe vaso-occlusive signs and a hyperhemolytic state. We report a 24-year-old male with sickle cell anemia (HbSS) who received 2 units of packed red blood cells for symptomatic anemia. Eight days post-transfusion, he presented with severe generalized bone pain and low-flow ischemic priapism lasting 24 hours. Laboratory assays revealed an acute drop in total hemoglobin from a post-transfusion peak of 9.4 g/dl to 3.8 g/dl, falling significantly below his steady-state baseline value of 6.2 g/dl. Total serum bilirubin rose to 7.8 mg/dl, and serum lactate dehydrogenase reached 1,850 U/L. A repeat direct antiglobulin test demonstrated strong complement (C3d) and IgG reactivity, and an antibody eluate assay identified anti-Jka alloantibodies that were undetectable during pre-transfusion screens. Intravenous methylprednisolone (1 g daily for 3 days) combined with high-dose intravenous immunoglobulin (1 g/kg for 2 days) stabilized the hemolysis and resolved the ischemic priapism without requiring surgical cavernous aspiration. Recognizing delayed immune hemolysis prevents further fatal transfusion episodes.

Keywords: Sickle cell anemia, delayed hemolytic transfusion reaction, hyperhemolysis, priapism, intravenous immunoglobulin

Manuscript Timeline: Received: December 14, 2016; Revised: January 20, 2017; Accepted: February 12, 2017; Published: March 18, 2017