Journal of Blood Medicine最新文献

We describe a case of refractory thrombotic thrombocytopenic purpura (7 lines of therapy) in which caplacizumab was used over a 6-month period as rescue therapy. Caplacizumab maintained the patient in clinical remission until successful immunosuppression was achieved resulting in normal ADAMTS13 levels. This case illustrates the utility of caplacizumab therapy in treating refractory TTP.

Background: The international consensus group suggested criteria for action following automated complete blood count and white blood cell differential analysis. These criteria were set based on data from laboratories of developed countries. It is highly important to validate the criteria in developing countries where infectious diseases are still rampant that can affect blood cell count and morphology. Thus, this study aimed to validate the consensus group criteria for slide review at Jimma Medical Center, Ethiopia from November 1, 2020 to February 30, 2021.

Methods: The study comprised a total of 1685 patient samples from the daily laboratory workload of CBC analysis. The samples were collected in K2-EDTA tubes (Becton Dickinson) and analyzed using Coulter DxH 800 and Sysmex XT-1880 hematology analyzers. A slide review was done on two Wright-stained slides for each sample. All statistical analyses were performed using SPSS version 20 software.

Results: There were 39.8% positive findings, the majority of which were related to red blood cells. The false negative and false positive rates for Sysmex and Coulter analyzer were 2.4% vs 4.8%; and 4.6% vs 4.7%, respectively. The false negative rate was unacceptably higher when we used physicians' triggered slide review, which was 17.3% and 17.9% for Sysmex and Coulter analyzers, respectively.

Conclusion: Generally, the consensus group rules are suitable to use in our setting. However, we might still need to modify the rules, particularly to reduce the review rates. It is also necessary to confirm the rules with case mixes proportionally derived from the source population.

Methemoglobinemia (MetHb) is a rare hematological condition characterized by high methemoglobin levels in the blood. It happens when hemoglobin is oxidized, resulting in hypoxia and cyanosis, which may occur in inherited or acquired forms. Inherited or congenital methemoglobinemia is a rare autosomal recessive condition and has never been reported in the Arab population. Here we report a case of a 22-year-old Arab man with a positive family history who presented with bluish discoloration of the fingers and lips and was found to have methemoglobinemia. Genetic study on the patient and his family revealed compound heterozygous variants in the CYB5R3 Exon 5 c.431G>A p.Gly144Asp likely pathogenic variant and CYB5R3 Exon 9 c.871G>A p.Val291Met variant of unknown significance. We suggest that the novel c.871G>A p.Val291Met variant could be causative for methemoglobinemia.

Background: Hematological abnormalities are a common complication of malaria infection. However, there is a paucity of evidence regarding it among malaria-infected adult patients in association with the ABO blood group in Ethiopia, particularly in the Harari Region. Therefore, this study aimed to assess the hematological abnormalities among malaria-infected adult patients in association with ABO blood groups at Jinella Health Center, Harar, Eastern Ethiopia.

Methods: An institutional-based cross-sectional study was conducted from July 10, 2022, to January 10, 2023. Four milliliters of venous blood were collected from each study participant. Drops of blood were used for blood film preparation. ABO blood group was determined by agglutination test using monoclonal anti-sera (Agape Diagnostics Ltd., India). A complete blood count was done using the DxH 800 (Beckman Coulter, Inc, Miami, FL) hematology analyzer. The data were analyzed using SPSS version 26. Bivariable and multivariable logistic regression models were fitted. The level of significance was declared at a p-value of <0.05.

Results: The study revealed that 47.2% (95% CI: 41.0 53.6) of the participants were anemic. Being female (AOR = 3.18, 95% CI = 1.67, 6.04), having the A blood group (AOR = 2.75, CI = 1.20, 6.31), and being infected with P. falciparum (AOR = 2.64, CI = 1.26, 5.53) were all significantly associated with malaria anemia. The overall prevalence of thrombocytopenia was also 67.7% (95% CI: 61.7-73.4%). It was significantly associated with P. falciparum infection (AOR = 8.03, CI = 3.53, 18.25) and high parasitemia levels (AOR = 4.40, CI = 1.57, 12.32).

Conclusion: Patients with malaria who belonged to the "A" blood group in the study area had anemia as a serious health problem. Hence, frequently checking for anemia in patients with malaria who have blood group "A" can help with early detection and better management of anemia.

Autoimmune hemolytic anemias (AIHAs) are rare and heterogeneous disorders characterized by the destruction of red blood cells by warm or cold antibodies. Hemolytic anemia associated with warm antibodies is the most common, whereas cold antibodies are rare and infrequent in cases published in the scientific literature. Herein, we present the case of a young patient with systemic lupus erythematosus (SLE) and autoimmune hemolytic anemia caused by cold antibodies. Initially, infectious etiology and hematological malignancy were considered, which were ruled out. She required management in the intensive care unit due to severe hematological involvement and responded well to immunomodulatory therapy. This case illustrates the importance of a strong clinical suspicion of AIHA due to cold agglutinins associated with SLE when faced with similar clinical symptoms in order to achieve a timely diagnosis and provide optimal therapy.

Introduction: Previous clinical trials established the efficacy and safety of sucrose-formulated recombinant factor (F) VIII (rFVIII-FS/Kogenate FS®/Helixate FS®) and octocog alfa (BAY 81-8973/Kovaltry®; LEOPOLD trials).

Aim: To report the results of a post hoc subgroup analysis assessing efficacy and safety outcomes in patients with hemophilia A who were receiving rFVIII-FS prior to enrolling into the LEOPOLD I Part B and LEOPOLD Kids Part A clinical trials and switching to octocog alfa.

Methods: LEOPOLD I Part B (NCT01029340) and LEOPOLD Kids Part A (NCT01311648) were octocog alfa Phase 3, multinational, open-label studies in patients with severe hemophilia A aged 12-65 years and ≤12 years, respectively. Annualized bleeding rate (ABR) was the efficacy endpoint for both studies. Safety endpoints included adverse events (AEs) and development of FVIII inhibitors.

Results: Of the 113 patients in both LEOPOLD trials, 40 (35.4%) patients received rFVIII-FS prophylaxis pre-study and had data available for pre-study total ABR. In LEOPOLD I Part B (n = 22, 35.5%), median (Q1; Q3) total ABR decreased from 2.5 (0.0; 9.0) pre-study to 1.0 (0.0; 6.8), and from 1.0 (0.0; 6.0) pre-study to 0.0 (0.0; 6.02) in LEOPOLD Kids Part A (n = 18, 35.3%). Octocog alfa was well tolerated, and no patients had drug-related serious AEs or inhibitors.

Conclusion: Treatment with octocog alfa prophylaxis appeared to have a favorable risk-benefit profile compared with rFVIII-FS and thus could be an effective and improved alternative strategy for individualized treatment for children, adolescent and adult patients with severe hemophilia A currently on rFVIII-FS treatment.

A 48-year-old patient with stable residual schizophrenia experienced a syndromic psychosis relapse following an episode of severe combined immunohemolytic and pure red cell aplastic anemia, with a hemoglobin level of 4.7 g/dl. The anemia was attributed to her anti-HIV medication zidovudine. Her HIV infection had been well-controlled; no other organic precipitant of the psychosis was found. Following transfusion of 2 units of leukocyte-poor packed red cells, schizophrenia symptoms promptly recovered to her baseline. This was maintained at 3- and 6-month follow-ups without any need for antipsychotic dose adjustment. Following zidovudine discontinuation and a short course of oral prednisolone, her anemia gradually recovered.

Primary cranial vault lymphoma (PCVL) is a rare lymphoma involving the skull with or without extra- and intracranial extension. Most cases of PCVL are diffuse large B-cell lymphoma (DLBCL). We report a case of primary cranial vault diffuse large B-cell lymphoma (PCV-DLBCL) that was successfully treated with anthracycline-based chemoimmunotherapy (CIT) alternating with central nervous system (CNS)-directed CIT with high-dose methotrexate and high-dose cytarabine. CNS-centric therapy was given for suspected cerebral cortical involvement and presumed elevated risk of CNS recurrence. The patient has remained in complete remission for 4.25 years following treatment. We suggest that PCV-DLBCL is potentially curable with CNS-directed therapy. Additionally, we provide genomic profiling results indicating an indeterminate cell of origin and multiple genetic mutations which are not frequently seen in DLBCL.

Purpose: To determine the prevalence of anemia and its association with Helicobacter pylori infection among adult dyspeptic patients.

Patients and methods: A cross-sectional study was conducted among 283 dyspeptic patients at Kiryandongo General Hospital, in Uganda. A structured questionnaire was administered to capture demographic and clinical characteristics of study participants. Four milliliters of blood were then collected into an EDTA vacutainer for Complete Blood Count (CBC) and analyzed using HUMA COUNT 30^TS, and peripheral blood smears were made and stained using Giemsa stain. Anemia was defined as hemoglobin levels <12g/dl in females and <13g/dl in men according to the World Health Organization (WHO). Helicobacter pylori (H. pylori) stool antigen test was performed using Whole power H. pylori Ag rapid test device, and saline stool preparation was examined for intestinal parasites. Chi-squared test and Logistic regression were performed to determine association, and a p-value of ≤0.05 was considered statistically significant.

Results: The overall prevalence of Helicobacter pylori infection was 42.4% (120/283). The prevalence of anemia among H. pylori-infected patients was 25.8% (31/120) and 15.3% (25/163) among H. pylori-negative counterparts. H. pylori infection was significantly associated with anemia (p-value 0.042), age (p-value 0.02, 0.009), water sources (p-value 0.0049,) and intestinal parasitic infestation (p-value 0.02), respectively.

Conclusion: This study has shown that the prevalence of H. pylori infection and anemia is high among dyspeptic patients at Kiryandongo General Hospital. H. pylori infection was found associated with anemia, age, water sources, and intestinal parasitic infestation. Routine screening of anemia in H. pylori-infected individuals and further studies to explore the relationship between anemia and H. pylori disease is highly recommended.

Purpose: In Norway, blood donors using antihypertensive medication were deferred until 2015. Following revision of the national directive, these donors could be allowed, providing stable dose for at least 3 months, adequate blood pressure control and no adverse effects caused by the therapy. The new practice was evaluated by a quality study where the major aim was to establish whether donations from blood donors on antihypertensive medication pose a risk to the donor. The risk was assessed by counting the number and categorizing the adverse events related to blood donation. In addition, the quantitative effect of including these donors was calculated.

Subjects and methods: In this retrospective quality study, blood donors on antihypertensive therapy were recruited from four different blood centers to fill out a questionnaire. A total of 265 donors answered questions regarding their health status, type of medication used, and adverse events connected to blood donation both before and after starting the therapy.

Results: No severe adverse events were observed in donors on antihypertensive medications. The amount of mild adverse events, as exhibited by only 7 persons (0.46%) in this donor population, was the same as for donors without hypertensive treatment.

Conclusion: Blood donation from persons on antihypertensive therapy poses no extra risk of severe adverse events, given the use of screening criteria to identify and bleed only low-risk donors.