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   Table of Contents - Current issue
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January-March 2022
Volume 13 | Issue 1
Page Nos. 1-70

Online since Thursday, April 28, 2022

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ORIGINAL ARTICLES  

Delta beta thalassemia, a rare hemoglobin variant: An experience from nodal centre in North Indian state Highly accessed article p. 1
Promil Jain, Nisha Marwah, Niti Dalal, Richa Pawar, Meenu Gill, Sanjay Kumar
DOI:10.4103/joah.joah_198_20  
CONTEXT: Fetal hemoglobin (HbF) reduces to <1% few months after birth. However, it can persist beyond infancy into adult life in rare conditions such as delta-beta (δβ) thalassemia and hereditary persistence of HbF. δβ thalassemia is a relatively rare type of thalassemia due to decrease in both beta and delta globin chain production. AIMS: This study aims to assess the hematological and high-performance liquid chromatography (HPLC) findings in δβ thalassemia. SETTINGS AND DESIGN: Cross-sectional observational study. SUBJECTS AND METHODS: The study was conducted in department of Clinical Pathology, PGIMS, Rohtak. On screening of 1197 patients over a period of around 1 year, a total of 13 cases of δβ thalassemia were detected by HPLC. STATISTICAL ANALYSIS USED: Not done. RESULTS: The age of presentation ranged from 1 to 42 years with a mean age of 22.8 years. Eleven cases were diagnosed as heterozygous δβ thalassemia and two as homozygous δβ thalassemia. CONCLUSIONS: δβ thalassemia is an uncommon cause of elevated HbF. Clinical and hematological parameters should be carefully analyzed for accurate diagnosis.
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Hematopoietic stem cell transplantation in paroxysmal nocturnal hemoglobinuria: Experience from a tertiary care center Highly accessed article p. 5
Duncan Khanikar, Sandip Shah, Akanksha Garg, Kinnari Patel, Kamlesh Shah, Aishwarya Raj, Harsha Panchal, Apurva Patel, Sonia Parikh
DOI:10.4103/joah.joah_15_21  
BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematological disorder characterized by episodic intravascular hemolysis. Despite newer therapies such as eculizumab, hematopoietic stem cell transplantation (HSCT) remains the only curative therapy. MATERIALS AND METHODS: An observational analytical retrospective study was conducted comprising eight PNH patients who had undergone HSCT at our center. From January 2014 to December 2020, we performed HSCT in 8 PNH patients, 5 female and 3 male, with median age of 32 years (range: 15–38 years). RESULTS: Seven patients had pancytopenia, associated with marrow hypocellularity. One patient presented with a thrombotic episode. Seven had matched sibling donor (MSD) transplant and one underwent haploidentical transplant. The median time from diagnosis to transplant was 7 months (range: 1–38 months). All received reduced intensity conditioning (RIC): Flu-Bu-Cy-A TG for MSD transplants and Flu-A TG-Post Cy in haploidentical transplants. Median CD34+ stem cell dose was 5.5 × 106/kg (range 3.4–5.8 × 106/kg). All had successful engraftment. Acute graft versus host disease (GVHD) Grade I–III occurred in six patients. Chronic GVHD occurred in two patients. One patient succumbed to Grade III liver GVHD. Rest seven patients are alive till date and transfusion independent. The overall survival was 87.5% at a median of 26 months. CONCLUSIONS: HSCT is the only curative therapy for PNH. With the use of RIC, the outcomes of PNH patients can be improved. The incidence of GVHD is high with RIC regimens but can be managed with prompt immunosuppression.
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Evaluation of pediatric anemia in rural population of Maharashtra, India Highly accessed article p. 9
Debopriya Chatterjee, Swapnil A More, Sneha R Joshi
DOI:10.4103/joah.joah_50_21  
INTRODUCTION: World Health Organization showed that the prevalence of anemia among children of 6–59 months was 42.6% globally in 2011 and 59% in India. Fourth National Family Health Survey, 2016, shows 58.6% of Indian children are anemic, of which 53.8% are in Maharashtra, especially among rural children. AIMS: The aim of the study was to study the morphologic and cytometric evaluation of anemia in pediatric population. MATERIAL AND METHODS: The study was conducted after obtaining approval from the ethics committee. All patients that came to outpatient department and inpatient department between 2016 and 2018, satisfying the inclusion criteria were included. Routine investigations performed were hemoglobin estimation, blood indices, and peripheral blood smear examination. RESULTS: The total number of pediatric patients that were found to be anemic was 400 (54.9%). Toddlers, aged 6 months to 6 years, were most anemic (48.0%). Moderate severity of anemia was seen most frequently (50.5%). Microcytic hypochromic anemia (67.0%), iron deficiency anemia was the most common cause seen (65.2%). CONCLUSION: The occurrence of anemia in children is essential to be constantly monitored to identify causes, formation of intervention strategies, and ensure already formed national programs are effective.
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Health-related quality of life of adolescents with sickle cell disease on hydroxyurea: A case-control study p. 13
Hayfaa Mohammed Mones, Meaad Kadhum Hassan, Bahaa Abd Al Hussein Ahmed
DOI:10.4103/joah.joah_7_21  
BACKGROUND: Sickle cell disease (SCD) is a chronic multisystem disorder associated with acute and chronic complications that may negatively impact the quality of life (QoL). The study aimed to assess the health-related QoL (HRQoL) of patients with SCD on hydroxyurea (HU) and the factors affecting HRQoL domains. MATERIALS AND METHODS: This case-control study included 174 patients with SCD (12–18-year-old); 87 were on HU for at least 1 year and 87 were not receiving HU. It also included 174 healthy adolescents of the same age group. The HRQoL was assessed using the Short Form 36 Health Survey version 2 (SF-36v2). A multivariate linear regression analysis was performed to assess the independent effect of studied variables on HRQoL dimensions. RESULTS: Patients with SCD who were not on HU were found to have significantly lower SF-36 v2 scores (all domains) compared to those on HU and control group, P < 0.001. While patients on HU had significantly lower SF-36 v2 scores in physical functioning, role physical (RP), general health, and physical health component score only compared to the control group, P < 0.001. Multivariate linear regression analysis revealed significant associations between duration of HU therapy and RP (R2 = 0.208, P = 0.021) and mental health component score (R2 = 0.389, P = 0.047) and between hemoglobin levels with social functioning (R2 = 0.370, P = 0.023). CONCLUSIONS: HU has improved the HRQoL of SCD patients, in almost all domains, in addition to the improvement in many disease-related complications, mainly painful episodes. The positive impact of HU on HRQoL was significantly associated with the duration of therapy.
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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 p. 22
Lobna Abdulaziz Baitalmal, Fawaz Abdulaziz Al Kasim, Eatidal Fathey Ghareeb, Fauzia Rehman Azmet, Parameaswari Parthasarathy Jaganathan
DOI:10.4103/joah.joah_244_20  
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
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Role of serum hepcidin and reticulocyte hemoglobin concentration in evaluation of anemia in ulcerative colitis patients p. 28
Samar Reda Ammar, Medhat A Ghazy, Maaly M Mabrouk, Amr M Gawaly
DOI:10.4103/joah.joah_127_21  
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.
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The prevalence of cumulative alloimmunization in patients with sickle cell disease at King Fahad University Hospital p. 35
Rabab Ahmad AlDawood
DOI:10.4103/joah.joah_122_21  
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.
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Maternal and neonatal variables affecting CD34+ cell count in the umbilical cord blood p. 41
Satya Prakash, Ashish Jain, Deepak Pahwa, Jaswinder Kaur Kalra, Rattiram Sharma
DOI:10.4103/joah.joah_68_21  
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.
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Iron deficiency anemia in pregnancy: Subgroup analysis from Riyadh mother and baby multicenter cohort study (RAHMA) p. 47
Hayfaa A Wahabi, Samia Esmaeil, Hala Elmorshedy, Hanadi Bakhsh, Aalaa Abdelrahman, Amel Fayed
DOI:10.4103/joah.joah_133_21  
OBJECTIVES: The objective of the study was to estimate the prevalence and risk factors of anemia among pregnant women in Riyadh and to examine its association with adverse pregnancy outcomes. METHODS: This study is a subgroup analysis from Riyadh mother and baby multicenter cohort study. Participants were grouped into four groups according to hemoglobin level: nonanemic (≥11.0 g/100 ml), mild (10–10.9 g/100 ml), moderate (7.0–9.9 g/100 ml), and severe anemia (<7.0 g/100 ml). Regression analyses were conducted to extrapolate the predicted probability (PP) for pregnancy outcomes. RESULTS: Out of 10,600 participants, 3261 (30.76%) were anemic; 1729 (16.3%), 1520 (14.3%), and 12 (0.1%) had mild, moderate, and severe anemia, respectively. The odds of anemia was higher in younger mothers (odds ratio [OR] = 0.94, confidence interval [CI]: 0.91–0.94) and in primiparous (OR = 1.01, CI: 0.90–1.14), while attendants of antenatal care and those who received iron supplements were less likely to be anemic (OR = 0.93, CI: 0.82–1.06) and (OR = 0.92, CI: 0.77–1.09), respectively. There was a significant increase in the odds of lower APGAR with the decreased hemoglobin; an increment of maternal hemoglobin by 1 g/100 ml decreased the likelihood of APGAR scores <7 by 9% (OR = 0.91, CI: 0.83–0.99). The PP showed a decrease in preterm birth (PTB) rate from 8% (CI: 6%–9%) to 6.5% (CI: 5%–8%), low birth weight (LBW) rate from 12% (CI 10%–13%) to 11% (CI: 9%–12%), stillbirth rate from 1.3% (CI: 0.7%–2.1%) to 1.1% (CI: 0.7%–1.6%), and maternal admission to intensive care unit (ICU) from 0.8% (CI: 0.2%–1.5%) to 0.2% (CI: 0.06%–0.4%), with increase of maternal hemoglobin from 7 g/100 ml to 15 g/100 ml. CONCLUSION: More than a third of the pregnant women in Riyadh had mild-to-moderate anemia. The odds of anemia increased in primipara, younger mothers, and those without antenatal care or iron supplementation. PP showed that anemia is associated with low APGAR scores, LBW, PTB stillbirth, and maternal admission to ICU.
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CASE REPORTS Top

Leukocyte adhesion defects Type III: A rare association of primary immunodeficiency and platelet functional defect p. 54
Anand Prakash
DOI:10.4103/joah.joah_43_21  
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.
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Linezolid-induced pancytopenia and hyponatremia p. 57
Satish Kumar, Narayan Dhakal, Vishal Mangal, Anil Menon
DOI:10.4103/joah.joah_29_21  
Linezolid (LnZ) is an antibiotic that is effective against Gram-positive bacteria and vancomycin-resistant enterococcus. LnZ is notorious to cause adverse hematological effects, primarily thrombocytopenia; however, pancytopenia is an infrequent complication. Euvolemic hyponatremia is a very rare adverse effect in patients receiving LnZ for more than 2 weeks. Early identification of pancytopenia and hyponatremia due to LnZ can result in avoidance of unnecessary investigations with improved patient outcome. An 80-year-old male with no previous comorbidities was admitted with a fracture neck of the left femur. He was empirically prescribed tablet LnZ 600 mg twice daily by the orthopedics team for soft-tissue infection over the sacral area. He underwent surgery for the left femur's fracture neck and was continued on LnZ in the postoperative period. Two weeks after the surgery, he was noticed to have pancytopenia and hyponatremia. After a thorough evaluation, LnZ was suspected to be the cause of pancytopenia and euvolemic hyponatremia. After 2 weeks of withholding LnZ, his pancytopenia and hyponatremia resolved. Physicians should be aware of the rare adverse effects of LnZ and should identify the patients at risk before administrating the drug. Regular monitoring of hematological and biochemical parameters is mandatory if intended to continue the drug for a more extended period.
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A rare clinical presentation of Hodgkin's disease p. 60
Prashanth Parameswaran, Dilip Harindran Vallathol, Narayanankutty Warrier, Sajna V Kutty
DOI:10.4103/joah.joah_234_20  
Hodgkin's disease or Hodgkin's lymphoma (HL) is a hematolymphoid neoplasm of B-cell lineage that has unique histologic, immunophenotypic, and clinical features. The occurrence of hemophagocytic lymphohistiocytosis (HLH) and neurological symptoms is rare in this disease. We present a case report of a 40-year-old woman who presented to our outpatient department with HLH and cerebellar signs. On further investigations, she was found to have classical HL. Her clinical condition improved with one cycle chemotherapy and steroids and she is on follow-up. This case indicates that a strong index of clinical suspicion is key to diagnose HLH and neurological symptoms (probably paraneoplastic) in HL. The cornerstone of management is identifying and treating the underlying cause.
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Atypical morphology and aberrant immunophenotypic expression: A diagnostic dilemma in acute promyelocytic leukemia p. 63
Shipra Verma, Paresh Singhal, Sharanjit Singh, Satyaranjan Das
DOI:10.4103/joah.joah_42_21  
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.
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IMAGE IN HEMATOLOGY Top

Root cause analysis of a moderately large fibrinous coagulum in a thawed fresh frozen plasma bag: Discussing the lessons learnt p. 68
Manish Raturi, Reshma Nambiyar, Yashaswi Dhiman, Adityaveer Sahrawat
DOI:10.4103/joah.joah_164_21  
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LETTER TO THE EDITOR Top

Bone marrow aspiration using 18g lumbar puncture needle: Correspondence p. 70
Rujittika Mungmunpuntipantip, Si M Sai Tin, Viroj Wiwanitkit
DOI:10.4103/joah.joah_178_21  
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