|Year : 2019 | Volume
| Issue : 1 | Page : 36-38
Infection with Plasmodium falciparum malaria in a patient with homozygous hemoglobin E
Prasanta Purohit1, Pradeep Kumar Mohanty2, Jogeswar Panigrahi3, Siris Patel1
1 Sickle Cell Institute, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Odisha, India
2 Department of Medicine, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Odisha, India
3 School of Life Sciences, Sambalpur University, Burla; Department of Biosciences and Bioinformatics, Khallikote University, Berhampur, Odisha, India
|Date of Web Publication||29-May-2019|
Dr. Siris Patel
Sickle Cell Institute, Veer Surendra Sai Institute of Medical Sciences and Research, Burla - 768 017, Odisha
Source of Support: None, Conflict of Interest: None
We present here a 22-year-old male with hemoglobin E (HbE) infected with severe Plasmodium falciparum malaria. The patient had acute renal failure with moderate anemia and thrombocytopenia. The patient was discharged on the 6th day of hospital admission after clinical recovery. To the best of our knowledge, this is the first report of a case with homozygous HbE and P. falciparum malaria in the state of Odisha, India.
Keywords: Hemoglobin E, malaria, Plasmodium falciparum
|How to cite this article:|
Purohit P, Mohanty PK, Panigrahi J, Patel S. Infection with Plasmodium falciparum malaria in a patient with homozygous hemoglobin E. Muller J Med Sci Res 2019;10:36-8
|How to cite this URL:|
Purohit P, Mohanty PK, Panigrahi J, Patel S. Infection with Plasmodium falciparum malaria in a patient with homozygous hemoglobin E. Muller J Med Sci Res [serial online] 2019 [cited 2019 Sep 18];10:36-8. Available from: http://www.mjmsr.net/text.asp?2019/10/1/36/259250
| Introduction|| |
Plasmodium falciparum malaria is a major public health problem in India with significant morbidity and mortality. The prevalence of many host genetic factors including hemoglobinopathies in malaria endemic regions provides a selective advantage against P. falciparum infection. Hemoglobin E (HbE), resulting from a single point mutation (Glutamic acid → Lysine) at 26th codon of the β-globin gene is found to be protective against severe malaria. We report a case of homozygous HbE (HbEE) with severe P. falciparum malaria in a malarial hyperendemic region of India.
| Case Report|| |
A 22-year-old male patient admitted with a history of fever to the Department of Medicine of Veer Surendra Sai Institute of Medical Sciences and Research (VSSIMSAR), Burla was screened for malaria and different hemoglobin variants at Sickle Cell Institute. Under a research activity, cases admitted to this institution with a history of fever were screened for P. falciparum infection and presence of different hemoglobin variants. The patient under consideration here was found to be positive for P. falciparum infection which was confirmed by polymerase chain reaction (PCR). Hemoglobin fractions studied by cation exchange high-performance liquid chromatography by using the VARIANT-II hemoglobin testing system; (Bio-Rad Laboratories, Hercules, CA, USA), resulting a high peak HbA2 window (82.5%) with HbA0 and HbF level of 2.9% and 2.0%, respectively [Figure 1]. Such high peak in the HbA2 window suggests the presence of various hemoglobin variants such as HbE, HbD-Iran, Hb Osu Christiansborg, and Hb Tianshui. The presence of HbE was confirmed by PCR followed by restriction fragment length polymorphism using Mnl1 as a restriction enzyme. The patient was found to be homozygous for HbE. Complete blood count was analyzed by Sysmex KX-21; Sysmex Corporation, Kobe, Japan. The total hemoglobin level was 9.6 g/dL with red blood cells of 4.7 million/μL and white blood cells of 6,800/μL of whole blood. The patient had microcytic (mean corpuscular volume, 62.1 fL) and hypochromic (mean corpuscular hemoglobin, 20.4 pg) red cells confirmed further by peripheral blood smear examination. Platelets count was 66,000/μL of whole blood. The liver function tests were in normal range whereas serum creatinine was elevated (3.1 mg/L). The raised creatinine in the patient implies the presence of acute renal failure as a phenotype of severe P. falciparum malaria. Alpha thalassemia was investigated by Gap-PCR and found to have a normal alpha globin genotype for both α−3.7 and α−4.2 deletion. Written informed consent was obtained from the case. This study was approved by Institutional Ethical Committee of VSSIMSAR.
|Figure 1: High-performance liquid chromatogram of the study case showing a high percentage of hemoglobin A2|
Click here to view
| Discussion|| |
HbE has a wide distribution throughout the Mediterranean, the Middle East, Southeast Asia, and the Indian subcontinent. In a multi-center study in India, the prevalence of HbE trait was found to be 3.63% with the majority were from North-Eastern Indian states such as Assam and West Bengal. In the state of Odisha, there were few reports on the prevalence of HbE as HbE trait, HbE, and HbE/β-thalassemia. The case presented here was a native of West Bengal and had come to western Odisha for job 2 years before attending to the hospital.
The state of Odisha is considered as a hyperendemic zone with low perennial transmission for malaria. It has been hypothesized that HbE retards intra-erythrocytic growth of P. falciparum and give protection against severe disease manifestation. Worldwide, there are several studies on the association of HbE and severity of P. falciparum malaria suggesting conflicting results.,,,, Although there are reports on the prevalence of HbE in India, no study has focused on its association with malaria. This is the first case in the state of Odisha presenting HbEE erythrocytes infected with P. falciparum malaria and is manifesting severe phenotypes of acute renal failure with moderate anemia and thrombocytopenia. The moderate anemia, in this case, might be due to the presence of HbEE because an individual with HbEE erythrocytes usually have a characterized mild anemia and microcytosis. This case study is important because instead of providing possible protection, the patient with HbEE presented with severe phenotypes of P. falciparum malaria. This might be due to the defective host immune mechanism against parasites or invasion of varied strains of parasites present in patient's working area which needs further investigation. However, the presence of dehydration state in the patient cannot be ignored as he had a history of fever and may have taken drugs to reduce temperature before attending to the hospital.
| Conclusion|| |
Screening of different hemoglobin variants including HbE in malaria endemic area will fill-up the knowledge gap with respect to the possible associations between them. Further, it may explore the facts concerning HbE as a “balanced polymorphism” in a different population of the Indian subcontinent.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
- Department of Science and Technology, New Delhi, Government of India
- Indian Council of Medical Research, New Delhi, Government of India.
Conflicts of interest
There are no conflicts of interest.
| References|| |
Thein SL, Lynch JR, Old JM, Weatherall DJ. Direct detection of haemoglobin E with mnlI. J Med Genet 1987;24:110-1.
Hill AV. Molecular epidemiology of the thalassaemias (including haemoglobin E). Baillieres Clin Haematol 1992;5:209-38.
Mohanty D, Colah RB, Gorakshakar AC, Patel RZ, Master DC, Mahanta J, et al.
Prevalence of β-thalassemia and other haemoglobinopathies in six cities in India: A multicentre study. J Community Genet 2013;4:33-42.
Balgir RS. Spectrum of hemoglobinopathies in the state of Orissa, India: A ten years cohort study. J Assoc Physicians India 2005;53:1021-6.
Nagel RL, Raventos-Suarez C, Fabry ME, Tanowitz H, Sicard D, Labie D, et al.
Impairment of the growth of Plasmodium falciparum
in HbEE erythrocytes. J Clin Invest 1981;68:303-5.
Santiyanont R, Wilairat P. Red cells containing hemoglobin E do not inhibit malaria parasite development in vitro
. Am J Trop Med Hyg 1981;30:541-3.
Vernes AJ, Haynes JD, Tang DB, Dutoit E, Diggs CL. Decreased growth of Plasmodium falciparum
in red cells containing haemoglobin E, a role for oxidative stress, and a sero-epidemiological correlation. Trans R Soc Trop Med Hyg 1986;80:642-8.
Hutagalung R, Wilairatana P, Looareesuwan S, Brittenham GM, Aikawa M, Gordeuk VR, et al.
Influence of hemoglobin E trait on the severity of falciparum malaria. J Infect Dis 1999;179:283-6.
Chotivanich K, Udomsangpetch R, Pattanapanyasat K, Chierakul W, Simpson J, Looareesuwan S, et al.
Hemoglobin E: A balanced polymorphism protective against high parasitemias and thus severe P falciparum
malaria. Blood 2002;100:1172-6.
Shannon KL, Ahmed S, Rahman H, Prue CS, Khyang J, Ram M, et al.
Hemoglobin E and glucose-6-phosphate dehydrogenase deficiency and Plasmodium falciparum
malaria in the Chittagong Hill districts of Bangladesh. Am J Trop Med Hyg 2015;93:281-6.
Vichinsky E. Hemoglobin E syndromes. Hematology Am Soc Hematol Educ Program 2007;2007:79 83.