International Journal of Hematology

International Journal of Hematology | Vol. 12, No. 1, January 2021 | pp. 1–8
DOI: 10.46882/2021/IJH/000131

Original Article

Title: Clinical relevance of serum soluble CD163 and ferritin kinetics as early markers of macrophage activation syndrome in adult-onset Still's disease

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

Abstract: Macrophage activation syndrome is a severe hyperinflammatory condition characterized by cytopenias, multi-organ dysfunction, and extreme hyperferritinemia. This prospective cohort study evaluated the diagnostic accuracy of monitoring serum soluble CD163 (sCD163) relative to hyperferritinemia kinetics in 36 adult patients with active adult-onset Still's disease suspected of developing macrophage activation syndrome. Serum soluble CD163 levels were quantified using an enzyme-linked immunosorbent assay. Mean serum ferritin concentrations were significantly elevated in patients with confirmed macrophage activation syndrome (16,450 ± 3,400 ng/ml). Soluble CD163 concentrations were also markedly elevated, with a mean value of 16.4 ± 3.8 μg/ml compared to 1.8 ± 0.5 μg/ml in active disease controls without macrophage activation (P < 0.001). Soluble CD163 levels correlated strongly with falling platelet counts and elevated serum triglycerides (r = 0.62, P < 0.01). Receiver operating characteristic curve analysis established that a soluble CD163 threshold above 7.2 μg/ml yielded a diagnostic sensitivity of 91.6% and a specificity of 88.8%. Measuring soluble CD163 serves as a highly specific biomarker for active macrophage activation, helping differentiate this hyperinflammatory storm from typical underlying disease flare-ups.

Keywords: Macrophage activation syndrome, ferritin, soluble CD163, hemophagocytosis, adult-onset Still's disease

Manuscript Timeline: Received: October 12, 2020; Revised: November 18, 2020; Accepted: December 05, 2020; Published: January 14, 2021

International Journal of Hematology | Vol. 12, No. 10, October 2021 | pp. 73–80
DOI: 10.46882/2021/IJH/000140

Short Communication

Title: Evaluation of automated microcytic cell mathematical indices in predicting latent iron deficiency in volunteer multiparous blood donors

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: Regular blood donations deplete maternal iron reserves, often inducing latent iron deficiency before total hemoglobin screening tests fall below acceptable donation thresholds. This diagnostic study evaluated the predictive performance of the Mentzer index and the Green and King mathematical cell counter formulas for identifying latent iron depletion in 120 regular multiparous female blood donors presenting with normal total hemoglobin levels (≥ 12.5 g/dl). Calculated indices were cross-validated against biochemical serum ferritin reference parameters. Latent iron deficiency, defined by a serum ferritin below 20 ng/ml, was confirmed in 24.1% (29 of 120) of the blood donor cohort. The Green and King formula achieved an isolated sensitivity of 89.6% and a positive predictive value of 76.1% for predicting depleted iron reserves, significantly outperforming the Mentzer index model (P < 0.05). Utilizing automated cell counter mathematical formulas offers an efficient, low-cost screening protocol to detect latent iron-restricted erythropoiesis and improve donor safety.

Keywords: Blood donors, latent iron deficiency, Mentzer index, Green and King formula, donor selection

Manuscript Timeline: Received: July 20, 2021; Revised: August 25, 2021; Accepted: September 14, 2021; Published: October 18, 2021

International Journal of Hematology | Vol. 12, No. 8, August 2021 | pp. 57–64
DOI: 10.46882/2021/IJH/000138

Original Article

Title: Evaluation of automated immature reticulocyte fraction and red blood cell fragmentation flags in separating iron deficiency from thrombotic thrombocytopenic purpura

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: Severe microcytic anemia fragments can mimic schistocytes on automated counters, requiring robust laboratory separation parameters to prevent inappropriate therapeutic decisions. This prospective diagnostic study evaluated the performance of automated immature reticulocyte fractions (IRF) and fragmented red blood cell (FRC) flags for separating absolute iron deficiency anemia from thrombotic thrombocytopenic purpura variants. Evaluations were conducted on 125 adult patients presenting with thrombocytopenia and microcytosis, and diagnoses were validated via serum ferritin, ADAMTS13 activity, and manual visual blood film schistocyte counts. Thrombotic thrombocytopenic purpura was confirmed in 50 cases (ADAMTS13 < 10%), while 75 presented with severe iron deficiency. The mean immature reticulocyte fraction was significantly higher in the thrombotic thrombocytopenic purpura cohort compared to the iron-deficient group (0.36 ± 0.08 versus 0.12 ± 0.03, P < 0.001), reflecting an intense bone marrow response. Conversely, automated fragmentation flags were elevated in both groups. Receiver operating characteristic analysis established a combined IRF and FRC model that achieved a diagnostic sensitivity of 92.8% and a specificity of 89.1% for identifying true microangiopathic processes. Utilizing automated reticulocyte maturity indices provides an efficient, low-cost asset for screening destructive hemolytic conditions.

Keywords: Immature reticulocyte fraction, fragmented red cells, iron deficiency anemia, schistocytes, cell counter indices

Manuscript Timeline: Received: May 12, 2021; Revised: June 20, 2021; Accepted: July 09, 2021; Published: August 14, 2021

International Journal of Hematology | Vol. 12, No. 5, May 2021 | pp. 33–40
DOI: 10.46882/2021/IJH/000135

Original Article

Title: Screening for lupus anticoagulant using a simplified textrin time and kaolin clotting time mixing protocol in unprovoked deep vein thrombosis

Names of Authors: Y. Z. Ibrahim¹, A. B. Okafor², C. D. Danjuma³

Authors’ Affiliations: ¹Department of Haematology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria; ²Department of Pathology, University of Ibadan, Ibadan, Nigeria; ³Department of Haematology, University of Maiduguri, Maiduguri, Nigeria

Abstract: Guidelines for diagnosing antiphospholipid syndrome require specific testing parameters that minimize false-positive results caused by circulating clotting factor variations. This prospective diagnostic study evaluated the performance of a combined screening protocol using the textrin time (TT) and the kaolin clotting time (KCT) mix to detect lupus anticoagulant in 70 adult patients presenting with acute, unprovoked lower limb deep vein thrombosis. Patient plasma underwent sequential 1:1 and 4:1 mixing loops with normal pooled plasma, followed by high-phospholipid corrections to calculate confirmatory ratios. Lupus anticoagulant presence was confirmed in 21.4% (15 of 70) of the thromboembolic cohort. The textrin-KCT dual mixing protocol achieved a diagnostic sensitivity of 91.4% and a specificity of 89.1% when cross-validated against international guidelines. Mixing indexes correlated positively with a clinical history of recurrent thrombotic events (r = 0.45, P < 0.05). Notably, the textrin-based step resisted interference from low-molecular-weight heparin due to its direct prothrombin activation pathway, providing a cost-effective and accurate thrombophilia risk-profiling asset for specialized clinical pathology laboratories.

Keywords: Lupus anticoagulant, textrin time, kaolin clotting time, mixing studies, deep vein thrombosis

Manuscript Timeline: Received: February 12, 2021; Revised: March 20, 2021; Accepted: April 05, 2021; Published: May 16, 2021

International Journal of Hematology | Vol. 11, No. 7, July 2020 | pp. 49–56
DOI: 10.46882/2020/IJH/000127

Case Report

Title: Spontaneous massive retroperitoneal hemorrhage secondary to acquired Factor VII inhibitor development in an elderly patient: Eradication with 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 directed against coagulation Factor VII is an exceptionally rare clinical condition that causes catastrophic bleeding events in elderly populations. We report a 74-year-old male who presented with sudden, unprovoked left flank pain, lower abdominal distension, and hypovolemic shock. Abdominal computed tomography confirmed a massive retroperitoneal hematoma measuring 12.4 × 8.5 cm without prior trauma or anticoagulant exposure. Coagulation profiles demonstrated isolated, severe prolongation of prothrombin time (52.4 seconds) with a normal activated partial thromboplastin time. A 1:1 mixing study with normal pooled plasma failed to correct the prothrombin time, indicating a specific extrinsic pathway inhibitor. Functional assays confirmed severely depressed Factor VII activity (< 1.0%), and a Bethesda assay quantified a Factor VII inhibitor titer of 24.5 Bethesda Units. Hemostasis was achieved using recombinant activated Factor VII bypassing agents (90 μg/kg every 3 hours) alongside supportive measures. Subsequent immunosuppressive therapy with oral prednisone paired with cyclosporine-A (3 mg/kg/day) cleared the inhibitor (0 BU) by week 8. This case demonstrates that acquired Factor VII autoantibodies can cause life-threatening retroperitoneal bleeding, requiring immediate diagnostic differentiation and multi-modal therapeutic strategies.

Keywords: Acquired factor VII inhibitor, retroperitoneal hemorrhage, extrinsic pathway, bypassing agents, cyclosporine

Manuscript Timeline: Received: April 18, 2020; Revised: May 22, 2020; Accepted: June 10, 2020; Published: July 16, 2020

International Journal of Hematology | Vol. 11, No. 6, June 2020 | pp. 41–48
DOI: 10.46882/2020/IJH/000126

Original Article

Title: Evaluation of baseline plasma protein C functional activity as an independent predictor of thromboembolic recurrence in active nephrotic syndrome

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: Massive renal wasting of natural anticoagulant factors establishes a profound hypercoagulable state in nephrotic disease, yet the clinical utility of functional protein C monitoring remains under-studied. This prospective study evaluated baseline plasma free protein C functional activity in 54 adult patients with active nephrotic syndrome to track its correlation with serum albumin depletion and 1-year thromboembolic recurrence outcomes. Protein C activity was quantified via chromogenic substrate assays prior to initiating immunosuppressive therapy. Severe protein C activity reduction (< 55.0%) was identified in 29.6% (16 of 54) of the nephrotic patients. Multivariable Cox proportional hazards analysis revealed that baseline protein C functional activity below 55.0% was an independent predictor of acute deep vein thrombosis or pulmonary embolism within a 12-month follow-up window (hazard ratio = 3.84, P < 0.01). Protein C depression correlated inversely with serum albumin levels (r = -0.65, P < 0.001) and positively with D-dimer concentrations. Screening for functional protein C profiles provides clear prognostic utility, helping identify high-risk nephrotic populations requiring early prophylactic anticoagulation.

Keywords: Nephrotic syndrome, protein C activity, hypercoagulability, albuminuria, thromboembolism

Manuscript Timeline: Received: March 14, 2020; Revised: April 25, 2020; Accepted: May 12, 2020; Published: June 19, 2020