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Introduction: Aplastic crisis is an uncommon complication during infection in any chronic hemolytic disease and it has been hardly found in a case of hemoglobin Constant Spring (Hb CS) disease.
Objective: To present a case of aplastic crisis in Hb CS disease during acute febrile illness due to influenza B infection.
Case Report: A 15-year-old Thai woman suddenly developed fever, cough, pallor, and malaise for 5 days. The physical examination revealed temperature 38.6 degree Celsius, and marked anemia with mild splenomegaly. Her blood tests revealed: Hb 6.5 g/dL, WBC 3.9 x109/L, N 54%, L 39%, M 7%, platelet 86 x109/L, MCV 77.7 fL, reticulocyte 0.7%. Hb analysis: Hb (CS)A2A, Hb A2 3.1%, Hb F 1.5%, the PCR tests for alpha thalassemia-1 and -2, and beta thalassemiagenes - all negative but positive for Hb CS genes mutation, ferritin 4,376 ng/mL, direct and indirect anti-globulin tests-negative, influenza type B-positive. She was diagnosed with aplastic crisis during fever, influenza type B and suspected Hb CS disease. She was immediately treated with blood transfusion, oseltamivir and supportive therapies. The fever subsided within two days and she was discharged with regularly oral folic acid and deferiprone therapy. She was well during follow-up every 3 months for four times. The successive blood tests were shown as mean: Hb 9.9 ± 0.3 g/dL, normal WBC and platelet, MCV 77.7 ± 2.0 fL, reticulocyte 5.6 %, ferritin 134.9 ng/mL.
Conclusion: Although a Hb CS disease is usually considered a mild hemolytic anemia, the aplastic crisis that needs the immediate transfusion during fever, can happen as seen in this case.
2. Chen P, Long GF, Lin WX, Li SQ. Homozygous hemoglobin constant spring in Guangxi province. Zhonghua Xue Ye Xue Za Zhi. 2004;25:205-8. [Article in Chinese]
3. Azma RZ, M-Gaus K, A-Aziz S, Alauddin H, Ithnin A, Razak NF, et al. Detection of homozygous haemoglobin Constant spring by capillary electrophoresis method. ARC J Hematol. 2016;1:28-32.
4. Derry S, Wood WG, Clegg JB, Weatherall DJ, Wikramasinghe SN, Darley J, et al. Hematologic and biosynthetic studies in homozygous Hemoglobin Constant Spring. J Clin Invest. 1984;73:1673-82.
5. Viprakasit V, Veerakul G, Sanpakit K, Pongtanakul B, Chinchang W, Tanphaichitr VS. Acute haemolytic crisis in a Thai patient with homozygous haemoglobin Constant Spring (Hb CS/CS): a
case report. Ann Trop Paediatr. 2004;24:323-8.
6. Charoenkwan P, Sirichotiyakul S, Chanprapaph P, Tongprasert F, Taweephol R, Sae-Tung R, et al. Anemia and hydrops in a fetus with homozygous hemoglobin Constant spring. J Pediatr
Hematol Oncol. 2006;28:827-30.
7. He Y, Zhao Y, Lou JW, Liu YH, Li DZ. Fetal anemia and hydrops fetalis associated with homozygous Hb Constant Spring (HBA2:c.427T>C). Hemoglobin. 2016;40:97-101.
8. Alavi S, Arabi N, Yazdi MK, Arzanian MT, Zohrehbandian F. Hereditary spherocytosis unmasked by human parvovirus B19 induced aplastic crisis in a family. Iran J Med Sci. 2015;40:461-4.
9. Jomoui W, Fucharoen G, Sanchaisuriya K, Nguyen VH, Fucharoen S. Hemoglobin Constant Spring among Southeast Asian Populations: Haplotypic heterogeneities and phylogenetic analysis. PLoS
ONE.2015; 10(12): e0145230. doi: 10.1371/journal.pone.0145230. PubMed PMID: 26683994; PMCID: PMC4686174
10. Rice J, Resar LMS. Hematologic abnormalities associated with influenza A infection: a report of 3 cases. Am J Med Sci 1998;316: 401-3.
11. Wang CC, Chen PY, Wang JD, Liu FC, Huang FL, Lee CY. Clinical and laboratory analysis of influenza B infection in children in Taichung, Taiwan during the 2006-2007 flu season. Pediatr
12. Shen CF, Huang SC, Wang SM, Wang JR, Liu CC. Decreased leukocytes and other characteristics of laboratory findings of influenza virus infections in children. J Microbiol Immunol Infect.
13. Rao KR, Patel AR, Anderson MJ, Hodgson J, Jones SE, Pattison JR. Infection with parvovirus-like virus and aplastic crisis in chronic hemolytic anemia. Ann Intern Med. 1983;98:930-2.
14. Krell S, Adams I, Arnold U, Kalinski T, Aumann U, Konig W, et al. Influenza B pneumonia with Staphylococcus aureus superinfection associated with parvovirus B19 and concomitant agranulocytosis.
15. Liebhaber SA, Schrier SL. Pathophysiology of α thalassemia. In: Steinberg MH, Forget BG, Higgs DR, Nagel RL, editors. Disorders of Hemoglobin; Genetics, Pathophysiology, and Clinical Management.
Cambridge: Cambridge University Press; 2001.391-404.
16. Schrier SL, Bunyaratvej A, Khuhapinant A, Fucharoen S, Aljurf M, Snyder LM, et al. The unusual pathobiology of hemoglobin Constant Spring red blood cells. Blood. 1997;89:1762-9.
17. Oh SE, Kim JH, Choi CH, Park KH, Jung JY, Park YI, et al. An adult with aplastic crisis induced by human parvovirus B19 as an initial presentation of hereditary spherocytosis. Korean J
Intern Med. 2005;20:96-9.