International Journal of Hematology

International Journal of Hematology | Vol. 5, No. 10, October 2014 | pp. 73–80
DOI: 10.46882/2014/IJH/000058

Original Article

Title: Characterization of erythrocyte morphologic parameters and osmotic fragility curves in hereditary spherocytosis variations

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: Hereditary spherocytosis is a heterogeneous hemolytic disorder caused by mutations in erythrocyte membrane proteins, resulting in reduced surface-area-to-volume ratios. This study characterized automated erythrocyte parameters and osmotic fragility profiles in 28 patients from 14 unrelated families presenting with chronic Coombs-negative hemolytic anemia. Full blood counts, peripheral film reviews, and standard erythrocyte osmotic fragility tests using fresh and incubated blood samples were performed. The mean corpuscular hemoglobin concentration (MCHC) was significantly elevated in hereditary spherocytosis patients compared to normal controls (36.2 ± 1.1 g/dl versus 32.8 ± 0.8 g/dl, P < 0.01). Osmotic fragility curves showed increased hemolysis in hypoosmolar saline solutions, which was pronounced after incubation at 37 °C for 24 hours. The initial lysis point occurred at a mean saline concentration of 0.65% in incubated spherocytosis blood compared to 0.44% in normal controls. An MCHC threshold above 35.5 g/dl combined with a positive incubated osmotic fragility test achieved a diagnostic sensitivity of 92.8%. Combining automated cell counter red cell parameters with traditional osmotic saline testing remains a reliable and accessible diagnostic protocol for confirming membrane defects in low-resource hematology centers.

Keywords: Hereditary spherocytosis, osmotic fragility, mean corpuscular hemoglobin concentration, hemolytic anemia, erythrocyte membrane

Manuscript Timeline: Received: July 12, 2014; Revised: August 20, 2014; Accepted: September 10, 2014; Published: October 14, 2014

International Journal of Hematology | Vol. 5, No. 8, August 2014 | pp. 57–64
DOI: 10.46882/2014/IJH/000056

Original Article

Title: Evaluation of zinc protoporphyrin levels as an adjunctive indicator for iron deficiency screening in prospective blood donors

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 Blood Transfusion, Lagos State University Teaching Hospital, Ikeja, Nigeria; ³Department of Chemical Pathology, University of Ilorin, Ilorin, Nigeria

Abstract: Screening blood donors solely via copper sulfate or hemoglobin hematocrit values can miss early, pre-anemic iron deficiency. This study evaluated the diagnostic performance of hematofluorometer-derived zinc protoporphyrin (ZPP) levels as a rapid screening tool for identifying subclinical iron deficiency in 250 prospective blood donors. Full blood counts and zinc protoporphyrin ratios were verified against gold-standard serum ferritin parameters. Subclinical iron deficiency, defined by a serum ferritin below 20 ng/ml despite a qualifying screening hemoglobin level (≥ 12.5 g/dl), was detected in 18.4% (46 of 250) of donors. The mean zinc protoporphyrin level was significantly higher in iron-deficient donors (68.4 ± 12.4 μmol/mol heme) compared to iron-replete individuals (32.2 ± 6.5 μmol/mol heme, P < 0.001). Receiver operating characteristic analysis established a zinc protoporphyrin threshold above 45.0 μmol/mol heme as optimal for predicting depleted iron stores, yielding a sensitivity of 84.8% and a specificity of 81.4%. Incorporating zinc protoporphyrin fluorometry into donor assessment protocols provides a fast and inexpensive diagnostic asset that prevents the induction of severe anemia in frequent blood donors.

Keywords: Blood donors, iron deficiency, zinc protoporphyrin, ferritin, pre-transfusion screening

Manuscript Timeline: Received: May 02, 2014; Revised: June 15, 2014; Accepted: July 05, 2014; Published: August 14, 2014

International Journal of Hematology | Vol. 4, No. 9, September 2013 | pp. 65–72
DOI: 10.46882/2013/IJH/000045

Original Article

Title: Coagulation profile and protein C activity variations in adult patients with acute 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: Determining baseline functional anticoagulant levels during an acute thrombotic episode helps optimize long-term thrombophilia risk evaluations. This study quantified plasma protein C activity, activated protein C resistance, and prothrombin time in 70 adult patients presenting with acute, Doppler-confirmed lower limb deep vein thrombosis prior to initiating anticoagulation. Plasma functional protein C activity was measured using a chromogenic substrate assay. A transient reduction in protein C activity (< 70%) was identified in 25.7% (18 of 70) of the acute patient cohort. Mean protein C activity during the acute phase was significantly lower (74.2 ± 12.4%) compared to measurements repeated 3 months post-thrombosis after completing warfarization (96.5 ± 11.2%, P < 0.01). True congenital protein C deficiency was confirmed in only 3 patients following familial evaluations and steady-state re-testing. Elevated D-dimer and consumption of natural anticoagulants during active clot propagation can lead to false-positive thrombophilia screens. These findings demonstrate that functional anticoagulant assays should be interpreted cautiously during an acute thrombotic event, and abnormalities should be confirmed after completing initial anticoagulant therapy.

Keywords: Deep vein thrombosis, protein C, thrombophilia, chromogenic assay, coagulation factors

Manuscript Timeline: Received: June 15, 2013; Revised: July 24, 2013; Accepted: August 11, 2013; Published: September 17, 2013

International Journal of Hematology | Vol. 4, No. 5, May 2013 | pp. 33–40
DOI: 10.46882/2013/IJH/000041

Original Article

Title: Clinical relevance of soluble CD163 and serum ferritin levels as biomarkers in macrophage activation syndrome

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, life-threatening complication of systemic inflammatory diseases characterized by cytopenias, hyperferritinemia, and multi-organ dysfunction. This prospective study evaluated the diagnostic performance of soluble CD163 relative to hyperferritinemia in 32 patients suspected of having macrophage activation syndrome secondary to systemic autoimmune disorders. Serum soluble CD163 levels were quantified using an enzyme-linked immunosorbent assay, and bone marrow aspirates were evaluated for hemophagocytosis. Mean serum ferritin concentrations were significantly elevated in patients with confirmed macrophage activation syndrome (14,250 ± 3,400 ng/ml). Soluble CD163 levels were also markedly elevated, with a mean value of 18.6 ± 4.2 μg/ml compared to 1.8 ± 0.5 μg/ml in active autoimmune controls without macrophage activation syndrome (P < 0.001). Soluble CD163 concentrations correlated strongly with the severity of cytopenias and elevated triglycerides (r = 0.64, P < 0.01). Receiver operating characteristic curve analysis demonstrated that a soluble CD163 threshold above 7.5 μg/ml yielded a diagnostic sensitivity of 92.5% and a specificity of 88.6%. Measuring soluble CD163 serves as a highly specific biomarker for active macrophage activation, helping differentiate this hyperinflammatory syndrome from flare-ups of underlying autoimmune conditions.

Keywords: Macrophage activation syndrome, ferritin, soluble CD163, hemophagocytosis, autoimmune disease

Manuscript Timeline: Received: February 10, 2013; Revised: March 18, 2013; Accepted: April 05, 2013; Published: May 14, 2013

International Journal of Hematology | Vol. 4, No. 3, March 2013 | pp. 17–24
DOI: 10.46882/2013/IJH/000039

Original Article

Title: Evaluation of a modified Matutes scoring system for the diagnosis of chronic lymphocytic leukemia

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: The Matutes scoring system distinguishes chronic lymphocytic leukemia from other mature B-cell lymphoproliferative disorders based on a five-marker immunophenotype. This study evaluated the diagnostic performance of a modified scoring system substituting CD200 for FMC7 in 50 patients presenting with persistent B-cell lymphocytosis. Multiparameter flow cytometry quantified expression of CD5, CD23, CD22, CD200, and surface light chain intensity. Chronic lymphocytic leukemia was confirmed in 38 cases based on clinical and histological features. The classic Matutes score (using FMC7) yielded a sensitivity of 89.4% and a specificity of 83.3%. In contrast, the modified score incorporating CD200 achieved a sensitivity of 97.3% and a specificity of 91.6% (P < 0.05). CD200 was strongly and uniformly expressed in all 38 chronic lymphocytic leukemia cases, whereas it was entirely absent or weakly expressed in mantle cell lymphoma and splenic marginal zone lymphoma cohorts. Incorporating CD200 into the immunophenotypic protocol significantly improves diagnostic accuracy, reducing borderline scores and helping differentiate chronic lymphocytic leukemia from more aggressive B-cell lymphomas.

Keywords: Chronic lymphocytic leukemia, Matutes score, CD200, flow cytometry, immunophenotyping

Manuscript Timeline: Received: December 05, 2012; Revised: January 14, 2013; Accepted: February 02, 2013; Published: March 12, 2013

International Journal of Hematology | Vol. 4, No. 11, November 2013 | pp. 81–88
DOI: 10.46882/2013/IJH/000047

Case Report

Title: Severe microangiopathic hemolytic anemia secondary to metastatic gastric adenocarcinoma mimicking thrombotic thrombocytopenic purpura

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: Cancer-associated microangiopathic hemolytic anemia is a rare, severe paraneoplastic syndrome that can clinically mimic thrombotic thrombocytopenic purpura, leading to diagnostic delays. We report a 45-year-old male presenting with severe fatigue, weight loss, and widespread petechiae lasting 3 weeks. Laboratory investigations revealed a microangiopathic hemolytic anemia profile: hemoglobin 5.4 g/dl, platelet count 18 × 10⁹/L, lactate dehydrogenase 2,150 U/L, and numerous schistocytes (8.5%) on peripheral blood film examination. Coagulation screens showed a normal prothrombin time and normal fibrinogen levels. Initial treatment for suspected thrombotic thrombocytopenic purpura with daily therapeutic plasma exchange was initiated, but it yielded no hematological or clinical improvement after 5 consecutive sessions. ADAMTS13 activity was subsequently reported as normal (68.0%), ruling out classic idiopathic thrombotic thrombocytopenic purpura. A subsequent bone marrow trephine biopsy revealed extensive infiltration by mucin-secreting signet-ring malignant cells. Upper gastrointestinal endoscopy confirmed a primary gastric adenocarcinoma. This case demonstrates that metastatic mucinous adenocarcinomas can present with severe microangiopathic hemolysis due to tumor-induced microvascular remodeling. Recognizing this diagnostic entity prevents ineffective plasma exchange and guides appropriate systemic palliative chemotherapy.

Keywords: Microangiopathic hemolytic anemia, gastric adenocarcinoma, schistocytes, thrombotic thrombocytopenic purpura, plasma exchange

Manuscript Timeline: Received: August 18, 2013; Revised: September 22, 2013; Accepted: October 12, 2013; Published: November 15, 2013