{"title":"Heinz body hemolytic anemia","authors":"V. Padma, A. Nair, S. Bhaskar","doi":"10.4103/joah.joah_163_20","DOIUrl":"https://doi.org/10.4103/joah.joah_163_20","url":null,"abstract":"Anemia is a common finding in our population. Females are highly predisposed to develop anemia. Evaluation of anemia due to a single cause may be straightforward in most of the situations, however rare causes cannot be ruled out. In this case report, we present to you a case of a 35-year-old female who came with complaints of breathlessness. On further evaluation, she had pallor, icterus, and mild splenomegaly. Her hemoglobin (Hb) was later found to be 8.1 g/dl, pyruvate kinase activity was normal, normal liver enzymes, Heinz body positive, and elevated retics count. Thus, she was found to have a rare form of anemia, i.e., Heinz body hemolytic anemia. She was advised for splenectomy and started on medications. Following the procedure, her Hb started to rise to 9.6 g/dl and reached to 13.8 in the following months.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"103 - 106"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48021126","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Pujani, C. Agarwal, Varsha Chauhan, Shivani Agarwal, Shanta Passi, Kanika Singh, Mita Singh
{"title":"Platelet parameters, neutrophil–lymphocyte ratio, platelet lymphocyte ratio, red cell distribution width: can they serve as biomarkers in evaluation of severity of psoriasis?","authors":"M. Pujani, C. Agarwal, Varsha Chauhan, Shivani Agarwal, Shanta Passi, Kanika Singh, Mita Singh","doi":"10.4103/joah.joah_195_20","DOIUrl":"https://doi.org/10.4103/joah.joah_195_20","url":null,"abstract":"BACKGROUND: Psoriasis has a multifactorial pathogenesis encompassing genetic, environmental, and immunological factors. There is a dire need for specific, cost effective, reliable, and universally accepted laboratory marker as indicator of severity of psoriasis. MATERIALS AND METHODS: A cross-sectional study was conducted on 50 psoriasis patients and 50 healthy controls. Hematological parameters including platelet indices (platelet count [PC], plateletcrit, mean platelet volume [MPV], platelet large cell ratio, platelet distribution width [PDW]), neutrophil–lymphocyte ratio (NLR), platelet–lymphocyte ratio (PLR), and red blood cell distribution width (RDW) were evaluated and correlation of these indices among themselves and with Psoriasis Area and Severity Index (PASI) analyzed. Statistical analyses were performed using SPSS version 19.0 (SPSS Inc., Chicago, IL, USA). RESULTS: A statistically significant difference in RDW, PC, MPV, PDW, NLR, and PLR was observed between psoriasis versus controls and mild versus moderate to severe psoriasis. There was a significant positive correlation between PASI and RDW, MPV, platelets, PLR while erythrocyte sedimentation rate showed a significant correlation with MPV. MPV and RDW, RDW and NLR, and RDW and PLR were also found to be correlated. MPV showed highest sensitivity and specificity both. MPV (area under the curve: 0.970, P < 0.001) demonstrated better predictive power as per area under curve of receiver-operator curve as compared to other parameters for psoriasis. CONCLUSION: The present study assessed the role of simple and low-cost parameters easily computed from routine tests like complete blood count as biomarkers for severity of psoriasis. Mean values of MPV, RDW, NLR, and PLR were found to be higher in psoriasis patients compared to controls. Moreover, a significant correlation was observed between PASI and these novel markers. However, additional large-scale, multicenter studies need to be conducted before application of these parameters in clinical practice.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"95 - 102"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"70811241","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lobna Baitalmal, F. Al kasim, EatidalFathey Ghareeb, Fauzia Azmet, P. Jaganathan
{"title":"Relationship between genotype variants and the age of first acute splenic sequestration in patients with sickle cell disease in a tertiary center of Saudi Arabia: A retrospective study","authors":"Lobna Baitalmal, F. Al kasim, EatidalFathey Ghareeb, Fauzia Azmet, P. Jaganathan","doi":"10.4103/joah.joah_244_20","DOIUrl":"https://doi.org/10.4103/joah.joah_244_20","url":null,"abstract":"BACKGROUND: Acute splenic sequestration crisis (ASSC) is one of the earliest life-threatening complications of sickle cell disease (SCD). Here, we aimed to identify the relation between the age of the first splenic sequestration episode and SCD variants. Episodes of acute splenic sequestration before 1 year of age are associated with a higher risk of recurrence. METHODS: We carried out a retrospective chart review study from the medical charts of SCD patients ≤12 years admitted to the Department of Pediatric Hematology King Saud Medical City, Riyadh with the first episode of ASSC and no other complications from January 2014 to December 2019. RESULTS: We included 47 patients (46.8% males, 53.2% females) diagnosed with SCD and having experienced their first ASSC in this review. The mean age of the patients at which the first episode of splenic sequestration happened was 3.26 years. The genotype distribution in the population was 74.5% HBSS, 21.3% HBS β0 thalassemia, and 4.3% HBSβ+ thalassemia. The crisis precipitated with fever in 74.5% of patients. Treatment included programmed blood transfusion (46.2%), splenectomy (29.8%). The recurrence rate after the first episode of ASSC was 59.6%. The high rate of recurrence was mainly due to the early age of presentation and poor compliance with suggested treatment. The use of hydroxyurea in 19.1% of patients showed a decrease of recurrence of ASSSC by 66.6% of patients. CONCLUSION: The study concluded that the HBSS variant of SCD had the earliest presentation with the first episode of ASSC but the differences were numerical without any statistical difference. Earlier age of presentation is associated with a higher rate of recurrence, but the difference was numerical not statistically significant. Our study shows hydroxyurea decreased recurrent splenic sequestrations; further research would help in establishing its role","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"22 - 27"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41379662","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S. Prakash, A. Jain, D. Pahwa, J. Kalra, Rattiram Sharma
{"title":"Maternal and neonatal variables affecting CD34+ cell count in the umbilical cord blood","authors":"S. Prakash, A. Jain, D. Pahwa, J. Kalra, Rattiram Sharma","doi":"10.4103/joah.joah_68_21","DOIUrl":"https://doi.org/10.4103/joah.joah_68_21","url":null,"abstract":"INTRODUCTION: Ease of collection, ready availability and lower graft-versus-host disease compared to peripheral blood stem cell favored umbilical cord blood stem cell transplantation. AIM: To assess the maternal and neonatal predictor affecting total nucleated and CD34+ cell count in cord blood collections. METHOD: A total of 200 Cord blood units were collected under aseptic conditions. Volume reduction was made by 6% hydroxyethyl starch followed by upright centrifugation of cord blood units to obtain a cellular pellet. Differential cell counts were done through hematology analyzer, viability testing by trypan blue exclusion test and percentage CD34, and CD45 estimation by flow cytometry. Two samples were HBsAg positive and not included in the study. RESULT: Total CD34 positive cell counts were more in cord blood units collected from younger age mother. Higher birth weight of newborns yielded a larger volume of cord blood unit and higher absolute TNC and CD34+ cell counts. The placental weight was positively correlated with the volume of cord blood collected, birth weight of new born, and total nucleated cell count but no correlation was observed with absolute CD34+ cell counts. The gravida status, gestational age and method of delivery were not significant with total nucleated cell (TNC), mononuclear cell (MNC), and CD34+ counts. CONCLUSION: Maternal age, birth weight, placental weight, and volume of CBUs were the most important predictor of getting increased nucleated and CD34+ cells in cord blood. Moreover, CBUs collected from pre-term deliveries were shown to have an approximately equal absolute number of CD34+ cells.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"41 - 46"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45439357","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The prevalence of cumulative alloimmunization in patients with sickle cell disease at King Fahad University Hospital","authors":"Rabab AlDawood","doi":"10.4103/joah.joah_122_21","DOIUrl":"https://doi.org/10.4103/joah.joah_122_21","url":null,"abstract":"BACKGROUND: Sickle cell disease (SCD) is caused by a mutation in the beta-globin gene. Red blood cell (RBC) transfusion is considered the mainstay of management. On the other hand, it carries many side effects, of which alloimmunization is the most significant. AIMS AND OBJECTIVES: The aim of this study is to identify the prevalence of alloimmunization among SCD patients and its relation with other independent risk factors in order to provide recommendations for the care of SCD patients. MATERIALS AND METHODS: This is a retrospective cohort study conducted at King Fahad Hospital of the University in Al-Khobar from January 1, 2010, to December 31, 2018. Data were collected from both the QuadraMed electronic system and the blood bank transfusion cards. Data were analyzed using IBM SPSS (version 23.0, Chicago, IL, USA). RESULTS: One hundred and seven out of 556 SCD patients developed alloantibodies with a prevalence of (19.2%). Anti-E was the most identified alloantibody in 37 (34.6%) patients, followed by anti-K in 33 (30.8%) of the alloimmunized SCD patients. We found a clinically significant correlation between the alloimmunization and age and direct antiglobulin test positivity. CONCLUSION: Alloimmunization is a major complication among SCD patients. Moreover, most of the formed alloantibodies were directed toward the Rh and K antigens. All SCD patients should undergo extended RBC phenotyping at the earliest opportunity, including the following RBC antigens (C/c, E/e, K, Jka/Jkb, Fya/Fyb, M/N, and S/s) at a minimum either serologically or by genotyping.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"35 - 40"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47080373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Shipra Verma, Paresh Singhal, Sharanjit Singh, Satyaranjan Das
{"title":"Atypical morphology and aberrant immunophenotypic expression: A diagnostic dilemma in acute promyelocytic leukemia","authors":"Shipra Verma, Paresh Singhal, Sharanjit Singh, Satyaranjan Das","doi":"10.4103/joah.joah_42_21","DOIUrl":"https://doi.org/10.4103/joah.joah_42_21","url":null,"abstract":"Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia, which is highly aggressive, yet the most curable. It is a medical emergency that requires a very high index of suspicion, as delay in the treatment could lead to fatality. The initial diagnosis is made on the basis of its typical clinical presentation, morphological and immunophenotypic features. However, definitive diagnosis rests on the cytogenetic study. We present one such interesting case of APL where the morphology as well as immunophenotypic features was quite deceiving, leading to a diagnostic dilemma.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"63 - 67"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41884203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Leukocyte adhesion defects Type III: A rare association of primary immunodeficiency and platelet functional defect","authors":"Anand Krishnan Prakash","doi":"10.4103/joah.joah_43_21","DOIUrl":"https://doi.org/10.4103/joah.joah_43_21","url":null,"abstract":"Leukocyte adhesion defect (LAD) Type III is an extremely rare disease, which presents with severe infections and a Glanzmann thrombasthenia-like bleeding phenotype. A 2-year-old male child with LAD Type III with recurrent mucosal bleeding and relatively few infections is presented to highlight this novel presentation. The rare association of primary immunodeficiency and platelet functional disease is described.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"54 - 56"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43127587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Role of serum hepcidin and reticulocyte hemoglobin concentration in evaluation of anemia in ulcerative colitis patients","authors":"Samar R Ammar, M. Ghazy, M. Mabrouk, A. Gawaly","doi":"10.4103/joah.joah_127_21","DOIUrl":"https://doi.org/10.4103/joah.joah_127_21","url":null,"abstract":"CONTEXT: One of the most common extra-intestinal signs of ulcerative colitis (UC) disease is anemia, which has a significant influence on patients' quality of life. AIM: The aim was to evaluate the role of serum hepcidin and reticulocyte hemoglobin concentration (CHr) in the study of anemia in UC patients. SUBJECTS AND METHODS: We recruited 80 UC patients and 30 healthy individuals of matched age and sex as controls. Subjects were subdivided into three groups – Group I: 50 anemic UC patients, Group II: 30 nonanemic UC patients, and Group III: 30 healthy controls. RESULTS: CHr showed a statistically highly significant decline in Group I than Groups II and III. Serum hepcidin showed a significant difference between Groups I, II, and III. We reported a significant negative correlation between CHr and severity of UC and extension of UC and a significant positive correlation between CHr and hemoglobin level, mean corpuscular volume (MCV), serum ferritin, and transferrin saturation. While, serum hepcidin had a significant negative correlation with severity and extension of UC and a significant positive correlation with hemoglobin level, MCV, serum ferritin, transferrin saturation, and CHr. CONCLUSIONS: CHr had an excellent performance in prediction of iron-restricted anemia and was the test of best performance in prediction of iron-deficiency anemia ± ACD. Serum hepcidin had an excellent performance in prediction of ACD.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"28 - 34"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43142606","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}