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   Table of Contents - Current issue
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October-December 2020
Volume 11 | Issue 4
Page Nos. 161-213

Online since Tuesday, November 17, 2020

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REVIEW ARTICLE  

Current status of stem cell transplantation in paroxysmal nocturnal hemoglobinuria p. 161
Akanksha Garg, Sandip Shah, Kamlesh Shah, Kinnari Patel
DOI:10.4103/joah.joah_51_20  
Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal, acquired hematopoietic stem cell disorder, which is caused by activation of the complement system leading to life-threatening manifestations such as hemolysis, thrombosis, and marrow failure. Eculizumab is a complement inhibitor of C5, which acts by blocking complement-mediated hemolysis. It needs to be administered lifelong to the patient; hence, there are major financial implications. This drug is easily available in the Western countries; however, in low resource countries, where its availability is limited, hematopoietic stem cell transplantation (HSCT) still remains the main modality for achieving cure in PNH. PNH being a rare disease, large prospective studies and guidelines are scarce. To choose the ideal candidate for transplant is the real challenge. This article aims to review the trends in HSCT for PNH, such as the use of reduced-intensity conditioning to attain the graft versus PNH effect or the use of haploidentical donors. In the era of complement inhibitor therapy, the role of transplantation still needs to be explored.
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ORIGINAL ARTICLES Top

Hematological, antioxidant, and trace elements status in healthy mechanical welders: A pilot study p. 169
Haladi Sudhir Prabhu, Faizan Kalekhan, Paul Simon, Prajna D'silva, Arnadi R Shivashankara, Manjeshwar Shrinath Baliga
DOI:10.4103/joah.joah_37_20  
BACKGROUND: Welding work is considered to be an occupational hazard and welders are exposed to a range of metal fumes that are toxic to the blood system. Regular inhalation of the welding toxic fumes alters the hematological, antioxidant, and trace element levels and therefore an attempt is made at understanding these changes in the welders. AIM OF THE WORK: In this case–control study, an attempt is made at understanding the general health, hematological, antioxidant, and trace elements status of welders by comparing with age-matched office workers from the same area. MATERIALS AND METHODS: This was a purposive, case–control prospective study and was carried out in healthy volunteers devoid of any chronic or acute systemic ailments in Mangalore, India. The sociodemographic details were collected in a structured questionnaire, while a detailed clinical examination was carried out by the senior clinicians. The blood collected as per the standard laboratory procedure was analyzed for hematological parameters, antioxidant, and trace elements status. The data were subjected to frequency, percentage, and analyzed using the unpaired ttest. P < 0.05 was considered statistically significant. RESULTS: The results suggest that when compared to the controls, the welders showed significantly lower neutrophil count (53.45 ± 6.11 vs. 46.68 ± 6.12; P = 0.0003) and platelet count (267409.1 ± 42329.4 vs. 199142.9 ± 73735.1; P = 0.0002), and significantly higher counts of eosinophils (5.86 ± 4.12 vs. 9.86 ± 2.76; P = 0.0004) and monocytes (2.45 ± 1.63 vs. 4.89 ± 1.17; P < 0.0001). The levels of lipid peroxidation were high (225.73 ± 56.88 vs. 255.82 ± 30.26; P = 0.04), whereas total antioxidant capacity was less (3.00 ± 0.91 vs. 2.16 ± 1.04; P = 0.004) in the welders. When compared to controls, the serum iron (84.09 ± 6.18 vs. 94.46 ± 8.44; P ≤ 0.0001), copper (104.68 ± 40.63 vs. 148.93 ± 34.18; P = 0.0002), and lead (8.53 ± 5.49 vs. 14.18 ± 8.05; P = 0.005) were all significantly high in welders. There was no significant difference in the serum zinc and glutathione levels between the controls and welders. CONCLUSIONS: The results of the study indicate that occupational exposure to welding fumes among welders disturbs the homeostasis of trace elements in systemic circulation and induces oxidative stress.
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Semi-nested polymerase chain reaction over blood culture in detection of bloodstream fungal infection in leukemic children with febrile neutropenia p. 174
Pradeep Paul, Nidhi Pandey, Vineeta Gupta, Vijai Tilak, Deepak Kumar, Ragini Tilak
DOI:10.4103/joah.joah_41_20  
BACKGROUND: Children with hematologic malignancies are currently at a severe threat of developing bloodstream fungal infection (BSFI). The early detection of BSFI followed by appropriate management of these patients remains a challenge to the oncologist of today. The conventional method of detection like blood culture followed by identification is time-consuming and of low accuracy. The molecular approaches like polymerase chain reaction (PCR) and its variants like semi-nested PCR (SnPCR) can be an attractive alternative to diagnose early and accurately BSFI but need to be explored. OBJECTIVE: The aim of our study was to determine the utility of SnPCR over conventional blood culture in detecting BSFI in leukemic children with febrile neutropenia. MATERIALS AND METHODS: A total of 53 pediatric patients with febrile neutropenia due to hematological malignancy were included in this study. From each patient, blood sample was collected for conventional blood culture, while SnPCR was performed from the overnight incubated blood broth. The blood culture-positive isolates were further identified to species level using conventional techniques. The PCR products obtained were sequenced and results interpreted after nucleotide BLAST and refinement. RESULTS: Fungal DNA was detected in 13 (24.5%) febrile neutropenic patients by SnPCR, while the conventional blood culture could detect only 6 (11.3%) fungemia cases. The SnPCR had a sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of 93%, 100%, 100%, 98%, and 98%, respectively, versus 43%, 100%, 100%, 83%, and 85%, respectively, for blood culture. The results by SnPCR alone showed almost perfect agreement with the results of the combined method (κ = 0.95) in the detection of BSFI. CONCLUSION: The SnPCR method proved to be better compared to blood culture for the early and accurate detection of BSFI in febrile neutropenic pediatric patients.
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Clinicohematological, treatment, and outcome profile for scrub typhus: Observations from a tertiary care center p. 180
Thomas George, Ramakrishna Pai Jakribettu, Soniya Abraham, Michael L J. Pais, Mohammed Adnan, Manjeshwar Shrinath Baliga
DOI:10.4103/joah.joah_54_20  
BACKGROUND: Scrub typhus, an acute febrile illness, is one of the emerging and re-emerging infectious diseases in India. This study was undertaken to assess the clinicohematological, treatment, and outcome profile of the patients diagnosed with scrub typhus at the clinical microbiology department of a tertiary care teaching hospital in Coastal Karnataka, India. MATERIALS AND METHODS: This was a retrospective study conducted with patients diagnosed as scrub typhus between January 2014 and December 2017. The demographic, clinical, laboratory, treatment, and outcome profile of these patients were noted and analyzed by Student's t-test. A P < 0.5 was considered statistically significant. RESULTS: A total of 146 patients were included in the study. The males were more infected than females, and people in the age group of 51–60 years were affected the most. Fever (139, 95.2%) was the most common symptom. Eschar was seen in only 52 (34.89%) patients. Anemia, leukocytosis, neutrophilia, lymphocytopenia, eosinophilia, monocytosis, thrombocytopenia, and raised erythrocyte sedimentation rate were statistically significant in scrub patients. The hepatic and renal indicators were also deranged. All patients were treated with doxycycline, and platelet was transfused in 7 (4.8%) patients. Among the seven dead patients, there were significant eosinopenia and deranged renal parameters compared to the survived patients. CONCLUSION: In endemic area, when a middle-aged individual presents with fever and leukocytosis with thrombocytopenia with or without eschar, scrub typhus should be considered as a differential diagnosis and empirical therapy started.
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Intravenous iron versus oral iron in anemia management for perioperative patients: A systemic review and meta-analysis p. 184
Ahmed H Alshantti, Zarour Ahmed, Sophie Robertson, Omar M Aboumarzouk, Alaa Alshantti
DOI:10.4103/joah.joah_76_20  
INTRODUCTION: Perioperative anemia commonly occurs in patients undoing major surgery. We aimed to assess the efficacy and safety of intravenous iron therapy (IVIT) given compared to standard practice of oral iron in the perioperative period. DESIGN: We conducted a Cochrane style systematic review. The search strategy included common search engines: Medline, Embase, Cochrane, and Google Scholar for only eligible clinical trials (randomized controlled trials) comparing IV over oral iron therapy up to July 2019. The primary outcome was the effect of IVIT on the change of hemoglobin level. The secondary outcomes were the effects of IVIT compared to oral iron on ferritin level, mean corpuscular volume, and adverse side effects. Data were collected from each trial and where applicable meta-analyzed using RevMan. RESULTS: Six randomized clinical trials that fit our inclusion criteria were included in the study. We found that IVIT increases the level of hemoglobin compared to oral iron (MD: Mean difference 0.90, 95% confidence interval [CI]: 0.44–1.36, P = 0.000). Serum ferritin levels increased significantly in favor of the IV iron group compared to the oral iron group at posttreatment (MD: 106.95, 95% CI: 73.29, 140.62 ng/mL). In addition, marked increases in the pooled mean corpuscular volume (MCV) level were observed in favor of the IVIT (MD: 6.07, 95% CI: −0.88–13.02). There was no significant difference in the rate of adverse effects in both the groups. CONCLUSION: IV iron therapy is more effective than oral iron on increasing hemoglobin, ferritin, and MCV in the perioperative period and is also as safe with no diffidence in the risk of developing adverse side effects.
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Green tea consumption effects on coagulation profile p. 191
Ahmed Mohammed Elnour Elkhalifa, Nada Yassin, Mohammed I Tabash, Albashir M M. Tom, Ebitsam H Msahad, Lina M H. Alnor, Mohammed Mohajar, Nahla Mahdi, Shadia Elnour, Mohammed A AL-Mohaithef
DOI:10.4103/joah.joah_77_20  
OBJECTIVES: The objective was to study the prothrombin time (PT) and activated partial thromboplastin time (APTT) values among Sudanese regularly drinking green tea. METHODS: This comparative study was conducted from May to November 2018 at the cities of Kosti, Rabak, and Sinnar, Sudan. Two hundred volunteers recruited in this study included individuals who drank more than three cups of green tea per day for at least 1 year or more (n = 100), and another group represented individuals who did not drink green tea (n = 100). Blood samples were collected in ethylenediaminetetraacetic acid and trisodium citrate anticoagulant containers. Coagulometer analyzer machine and standard methods were used for PT and APTT analysis. RESULTS: The study results showed significantly prolonged in both PT and APTT mean values of individuals who drank green tea when compared with those who did not drink green tea. The PT mean values are 19.88 ± 3.7 s vs. 15.43 ± 0.52 s (P < 0.001) and the APTT mean values are 34.94 ± 3.4 s vs. 32.86 ± 3.2 s (P < 0.002). The multiple linear regression analysis confirmed a positive impact relationship between age, drinking green tea, and duration time of drinking green tea with both PT and APTT values. CONCLUSION: The study concludes that daily drinking green tea for a long time could affect an individual's coagulation profile.
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CASE REPORTS Top

Resolution of the complexity of transfusion support by alloadsorption in a patient of thalassaemia intermedia with multiple alloantibodies p. 195
Gopal Krushna Ray, Somnath Mukherjee, Dibyajyoti Sahoo, Debasish Mishra, Suman Sudha Routray, Satya Prakash
DOI:10.4103/joah.joah_23_20  
Thalassemia intermedia (TI) patients are generally more prone to alloimmunization against red blood cell antigen. Laboratory diagnosis of multiple alloantibodies faces challenges due to history of recent transfusion, lack of infrastructure, and skilled workforce. Alloadsorption is commonly used for a recently transfused patient suspected of having multiple alloantibodies. In this case, alloadsorption was performed with rr cells only to rule out the presence of allo-anti-E. Blood transfusion is the mainstay of treatment, and transfusion dependence is the primary measure for determining the severity. A 12-year-old young female patient, already diagnosed case of TI, presented with anemia, jaundice, and abdominal pain (on ultrasonography, hepatosplenomegaly and cholelithiasis) and planned for splenectomy with cholecystectomy. Cross-match was incompatible with the group-specific A-positive red cell unit in the antihuman globulin phase. Antibody screening and antibody identification (AI) revealed the possibility of anti-c and anti-E. Repeated alloadsorption with c+E-(rr) red cells, and further AI with remaining serum, confirmed the presence of both anti-c and anti-E. The patient was transfused with two units of c and E antigen-negative, A-positive compatible unit and operated without any transfusion-related adverse event. Here, we depicted the importance of alloadsorption as a tool to resolve multiple alloantibodies.
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Detection of anti-f on a 51-year-old Saudi female p. 199
Ahmed Alharbi, Khalid Batarfi, Paolo Domingo Kiseo, Jabree Faten Saleh, Ahmed Ali Shareefi, AL-Harthi Nourah
DOI:10.4103/joah.joah_59_20  
Anti-f/ce is a rare antibody but nevertheless associated with Hemolytic Disease of the Fetus and Newborn (HDFN) and Hemolytic Transfusion Reactions (HTR). This antibody shows reaction against products of the seemingly common R0 or r haplotypes. Often detected with other Rh antibodies, these reactions are enhanced against cells pretreated with common protease enzymes. The findings concluded the presence of anti-f/ce using routine procedures. Although seemingly innocuous being 'enzyme only', cases as these must be dealt with proper discernment to avoid risks of HTR and HDFN. The purpose of this article is to report the detection of an anti-f on a 51-year-old female with unknown transfusion history by a routine blood bank and to show how these infrequent antibodies are being misdiagnosed which could be avoided.
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Management of polycythemia vera by endoscopic parathyroidectomy p. 204
R Padmakumar, Kevin J Chiramel, Madhukara Pai, Farish Shams, Premna Subin
DOI:10.4103/joah.joah_61_20  
Polycythemia vera (PV) is a hemotologic disease. Majority of persons with PV, essential thrombocythemia and primary myelofibrosis show the Janus kinase (JAK) 2 gene– which is needed for the normal development of blood cells. PV is generally controlled with medication: hydroxyurea or pipobroman. Phlebotomy is the mainstay of therapy for PV. Association between hyperparathyroidism and PV has been very sparingly considered. Primary hyperparathyroidism may produce a growth factor, which induces pancytosis, especially in the presence of high levels of ionized calcium. It is found that parathyroid hormone levels (PTH) could influence hemopoiesis through a direct action on hemopoietic precursors. A 52-year-old female presented with complaints of fever associated with headache and generalized weakness. On evaluation, her hemoglobin value was found to be high (Hb– 18.8 g/dl) with leukocytosis. She was evaluated for polycythemia, which showed JAK-2 mutation positivity. She was started on Enteric Coated Aspirin. She was managed initially with twice-weekly venesection to keep packed cell volume <45. It was observed that she was having very high PTH– 569.9 pg/ml and low Vitamin D levels– 8.41 mg/ml. She underwent a Technetium 99M Sestamibi Scan (TcMIBI), which showed a large adenoma involving the left inferior parathyroid gland. She was taken up for endoscopic parathyroid removal through the left axillo-breast approach. Intraoperatively, PTH value had dropped from 569.9 pg/ml to 62 pg/ml. Hemoglobin level decreased to normalcy by the 2nd week of surgery without any medication or venesection. Patients with polycythemia must be screened for hyperparathyroidism. Early treatment of hyperparathyroidism by parathyroid removal will take care of polycythemia as well. Endoscopic parathyroidectomy is very feasible, safe, and well accepted by patients.
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Disease in disguise – An unusual cause of leukocytosis in an infant – A case report and review of the literature p. 208
Meenakshi Mohan, Dhaarani Jayaraman, Sri Gayathri Shanmugam, Julius Xavier Scott
DOI:10.4103/joah.joah_85_20  
Leukocytosis with cytopenias of other cell lines and organomegaly in children pose a significant diagnostic challenge. Leukemoid reaction is exaggerated leukocytosis, whereas misinterpretation of nucleated red blood cells (nRBC) leads to spurious leukocytosis. These need to be considered in given clinical context to investigate further appropriately. We describe a report of 6 months infant who was referred with suspected acute leukemia based on initial evaluation when he presented with severe pallor and hepatosplenomegaly with white blood cell >50× ×109/L. He was later found to have spurious leukocytosis with an underlying hemolytic anemia. The strikingly normal platelet count, absence of blasts in smear and low mean corpuscular volume for age gave initial clues away from leukemia. This case emphasizes the need for systematic approach to clinical features followed by meticulous analysis of every parameter of hemogram and peripheral smear to arrive at appropriate diagnosis and to avoid unnecessary investigations.
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IMAGE IN HEMATOLOGY Top

Disseminated cryptococcal infection involving bone marrow p. 211
Vishal Mangal, Anurag Singh, Manish Manrai
DOI:10.4103/joah.joah_56_20  
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LETTER TO THE EDITOR Top

Depression among patients with immune thrombocytopenia: Are we missing the elephant in the room? p. 212
Anwar A Sayed
DOI:10.4103/joah.joah_138_20  
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