Literature DB >> 24339473

Hemophagocytic lymphohistiocytosis in an infant with probable HIV infection.

Drishti Tolani1, Ira Shah.   

Abstract

Entities:  

Year:  2013        PMID: 24339473      PMCID: PMC3841674          DOI: 10.4103/0253-7184.120579

Source DB:  PubMed          Journal:  Indian J Sex Transm Dis AIDS        ISSN: 2589-0557


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Sir, 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 6th 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/mm3 (50% polymorphs, 48% lymphocytes), platelets = 85,000/mm3), 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. CD4 count was 930 cells/mm3 with CD4:CD8 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.
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1.  Profile of hemophagocytic lymphohistiocytosis in children in a tertiary care hospital in India.

Authors:  B Ramachandran; S Balasubramanian; N Abhishek; K G Ravikumar; A V Ramanan
Journal:  Indian Pediatr       Date:  2010-08-01       Impact factor: 1.411

Review 2.  Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives.

Authors:  James W Verbsky; William J Grossman
Journal:  Ann Med       Date:  2006       Impact factor: 4.709

Review 3.  Haemophagocytic syndrome in patients infected with the human immunodeficiency virus: nine cases and a review.

Authors:  G Grateau; C Bachmeyer; P Blanche; M Jouanne; M Tulliez; C Galland; D Sicard; D Séréni
Journal:  J Infect       Date:  1997-05       Impact factor: 6.072

4.  Successful recovery from human immunodeficiency virus (HIV)-associated haemophagocytic syndrome treated with highly active anti-retroviral therapy in a patient with HIV infection.

Authors:  M Gotoh; J Matsuda; K Gohchi; T Sanaka; K Kawasugi
Journal:  Br J Haematol       Date:  2001-03       Impact factor: 6.998

5.  Acquired immunodeficiency syndrome. Clinicopathologic study of 56 autopsies.

Authors:  G W Niedt; R A Schinella
Journal:  Arch Pathol Lab Med       Date:  1985-08       Impact factor: 5.534

6.  Functional consequences of perforin gene mutations in 22 patients with familial haemophagocytic lymphohistiocytosis.

Authors:  Jérôme Feldmann; Françoise Le Deist; Marie Ouachée-Chardin; Stéphanie Certain; Sarah Alexander; Pierre Quartier; Elie Haddad; Nico Wulffraat; Jean Laurent Casanova; Stéphane Blanche; Alain Fischer; Geneviève de Saint Basile
Journal:  Br J Haematol       Date:  2002-06       Impact factor: 6.998

7.  Acute human immunodeficiency virus syndrome presenting with hemophagocytic lymphohistiocytosis.

Authors:  Kyung-Hwa Park; Ho-Sung Yu; Sook-In Jung; Dong-Hyeon Shin; Jong-Hee Shin
Journal:  Yonsei Med J       Date:  2008-04-30       Impact factor: 2.759

  7 in total

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