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Year : 2013  |  Volume : 34  |  Issue : 2  |  Page : 152-153

Hemophagocytic lymphohistiocytosis in an infant with probable HIV infection

Pediatric HIV Clinic, Department of Pediatrics, Bai Jerbai Wadia Hospital for Children, Mumbai, India

Date of Web Publication25-Oct-2013

Correspondence Address:
Ira Shah
1/B Saguna, 271/B St Francis Road, Vile Parle (W), Mumbai - 400 056
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0253-7184.120579

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How to cite this article:
Tolani D, Shah I. Hemophagocytic lymphohistiocytosis in an infant with probable HIV infection. Indian J Sex Transm Dis 2013;34:152-3

How to cite this URL:
Tolani D, Shah I. Hemophagocytic lymphohistiocytosis in an infant with probable HIV infection. Indian J Sex Transm Dis [serial online] 2013 [cited 2020 Jun 4];34:152-3. Available from:


A 2-month-old boy born to Human Immunodeficiency Virus (HIV) infected parents presented with fever, cough, breathlessness, and refusal of feeds for 8 days. Parents were detected to be HIV infected during 6 th month of gestation. However, no antiretroviral therapy (ART) was given to the mother and she delivered the baby vaginally at 40 weeks of gestation. Birth weight was 3.0 kg and child was given breast feeds. On examination, heart rate was 130/min, respiratory rate was 30/min, and chest was clear. There was hepatosplenomegaly with pallor. Investigations showed pancytopenia (hemoglobin = 7.5 gm/dl, white blood cells (WBCs) =4,900/mm 3 (50% polymorphs, 48% lymphocytes), platelets = 85,000/mm 3 ), with erythrocyte sedimentation rate of 56 mm at end of 1 h. Peripheral smear showed presence of hemophagocytes and bone marrow examination showed poor erythropoiesis with occasional hemophagocytes with fibrosis. Serum fibrinogen was 200 mg/dl (normal: 150-200 mg/dl) and triglycerides were 148 mg/dl (normal: <200 mg/dl). D-dimers were >3,000 and liver function tests were normal. CD 4 count was 930 cells/mm 3 with CD 4 :CD 8 ratio of 2:1. HIV DNA PCR test was advised; however child went discharge against medical advice. Though our patient did not have hypofibrinogenemia and hypertriglyceridemia, he had hemophagocytes along with fever, splenomegaly and cytopenia suggestive of hemophagocytic lymphohistiocytosis (HLH).

HLH is a potentially fatal disease in children with an annual incidence of approximately 1.2 million cases. [1] Death usually occurs if prompt treatment is not given. [2] Cases of HIV-associated HLH are being increasingly reported [3] but most of these involve late stages in HIV infection. [4] An autopsy study of HIV patients has revealed that nearly 20% of HIV patients have associated hemophagocytosis. [5] Rarely is HLH diagnosed during the acute or seroconversion stage of AIDS. [3] Antiretroviral therapy (ART) in cases of HLH with HIV has proved to be successful. [6] In India, cases of HLH associated with perinatal tuberculosis, dengue fever, Epstein-Barr virus (EBV), cytomegalovirus (CMV), and bacterial agents have been reported in children but rarely or rather never has a case of HIV associated with HLH been reported. [7] Our patient was born to HIV positive parents. Moreover, the mother did not receive ART during pregnancy and breast fed him after delivery increasing the risk of of transmission of HIV to the child. However, whether the child was HIV infected or not, could not be established and was suspected to have probable HIV infection based on his clinical presentation.

Thus, it can be concluded that HLH associated with probable HIV infection can occur in infants.

   References Top

1.Verbsky JW, Grossman WJ. Hemophagocytic lymphohistiocytosis: Diagnosis, pathophysiology, treatment, and future perspectives. Ann Med 2006;38:20-31.  Back to cited text no. 1
2.Feldmann J, Le Deist F, Ouachée-Chardin M, Certain S, Alexander S, Quartier P, et al. Functional consequences of perforin gene mutations in 22 patients with familial haemophagocytic lymphohistiocytosis. Br J Haematol 2002;117:965-72.  Back to cited text no. 2
3.Park KH, Yu HS, Jung SI, Shin DH, Shin JH. Acute human immunodeficiency virus syndrome presenting with hemophagocytic lymphohistiocytosis. Yonsei Med J 2008;49:325-8.  Back to cited text no. 3
4.Grateau G, Bachmeyer C, Blanche P, Jouanne M, Tulliez M, Galland C, et al. Haemophagocytic syndrome in patients infected with the human immunodeficiency virus: Nine cases and a review. J Infect 1997;34:219-25.  Back to cited text no. 4
5.Niedt GW, Schinella RA. Acquired immunodeficiency syndrome. Clinicopathologic study of 56 autopsies. Arch Pathol Lab Med 1985;109:727-34.  Back to cited text no. 5
6.Gotoh M, Matsuda J, Gohchi K, Sanaka T, Kawasugi K. Successful recovery from human immunodeficiency virus (HIV)-associated haemophagocytic syndrome treated with highly active anti-retroviral therapy in a patient with HIV infection. Br J Haematol 2001;112:1090.  Back to cited text no. 6
7.Ramachandran B, Balasubramanian S, Abhishek N, Ravikumar KG, Ramanan AV. Profile of hemophagocytic lymphohistiocytosis in children in a tertiary care hospital in India. Indian Pediatr 2011;48:31-5.  Back to cited text no. 7


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