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Indian Pediatr 2014;51: 496 -497 |
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Dengue Associated Hemophagocytic
Lymphohistiocytosis: A Case Series
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Priyankar Pal, Prabhas Prasun Giri and *AV Ramanan
From Departments of Pediatric Rheumatology, Institute
of Child Health, Kolkata, India and
*Bristol Royal Hospital for Children, UK.
Email:
[email protected]
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Hemophagocytic lymphohistiocytosis is a rare complication of dengue. We
present 8 cases of dengue associated hemophagocytic lymphohistiocytosis
diagnosed in our hospital during the dengue outbreak of 2012. All the
cases were treated with a short (4 weeks) course of steroids along with
supportive measures, and showed an excellent response.
Keywords: Dengue fever, Hepatomegaly,
Splenomegaly.
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Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of
clinical syndromes characterised by activation and subsequent
uncontrolled non-malignant proliferation of T-lymphocytes and
macrophages, leading to a cytokine storm. The clinical features are:
fever, hepatosplenomegaly, multiorgan dysfunction and fulminant
pancytopenia resembling severe sepsis [1]. Persistence of fever for more
than 7 days, with persistent/progressive cytopenias, raised ferritin,
and organomegaly suggested the possibility of HLH in our patients with
dengue.
Clinical records of children (<15 years) with
serologically confirmed dengue infection admitted at the Institute of
Child Health, Kolkata during the Dengue outbreak (July 2012 to November
2012) were reviewed. Out of 358 dengue, 8 developed HLH. The diagnosis
of HLH was based on the criteria laid down by the Histiocytic Society in
2004. The clinical and laboratory features are listed in Table
I.
TABLE I Clinical and Laboratory Features in the Study Children
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Clinical Parameters |
No. (%) |
Laboratory Parameters |
No. (%) |
Range |
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Fever >7 d
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8 (100%) |
Anemia (<9 g/dL) |
7 (88%) |
6.7-9.2 |
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Rash/mucositis |
5 (63%) |
Thrombocytopenia (<100000/cmm) |
8 (100%) |
8.8-9.6 |
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Hepatomegaly |
8 (100%) |
Neutropenia (ANC<1000) |
6 (75%) |
352-1622 |
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Splenomegaly |
7 (88%) |
Raised CRP (>6 mg/L) |
8 (100%) |
82-216 |
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Bleeding manifestations |
6 (75%) |
Raised SGPT (>50IU/L) |
7 (88%) |
42-288 |
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Lymphadenopathy |
2 (25%) |
Raised Ferritin (>500 ng/L) |
8 (100%) |
1832-64,600 |
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Edema |
8 (100%) |
Raised LDH (>500U/L) |
8 (100%) |
872-2680 |
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Ascites |
8 (100%) |
Raised d-dimer (>1000) |
8 (100%) |
6800->10,000 |
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Pleural effusion |
8 (100%) |
Raised triglyceride (>265 mg/dL) |
7 (88%) |
242-638 |
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ARDS |
2 (25%) |
Decreased fibrinogen (<1.5 g/dL) |
5 (63%) |
86-220 |
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Myocarditis |
4 (50%) |
Altered PT/APTT |
8 (100%) |
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Hypotension |
6 (75%) |
Hemophagocytes in bone marrow |
6 (75%) |
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Encephalopathy |
2 (25%) |
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Joint pain |
2 (25%) |
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The children were given supportive therapy in form of
blood component transfusions, as and when required, along with broad
spectrum antibiotics. Definitive therapy was administered in the form of
parenteral steroids (dexamethasone 10 mg/m 2
in 3-4 divided doses/day) and continued till child was hemodynamically
stable or accepting oral feeds. It was switched to oral dexamethasone in
a in a tapering dose for 21 d (5 mg/m2
for next 7 days and then 2.5 mg/m2
for further 7 days, and ultimately tapered off over the next 7 days). In
seven children, fever subsided within 48-72 h of starting steroids;
reversal of cytopenias and regression of hepato-splenomegaly occurred
over the next 4-7 days. Serum ferritin started normalizing within a
week. Intravenous immunoglobulin was used (1 g/kg) in one patient as a
rescue measure as the child did not show any significant improvement
after 48 hours of starting steroids.
HLH is a potentially life-threatening condition with
protean clinical manifestations [2]. HLH secondary to infections (IAHLH)
is the commonest, especially in tropical countries. Any infection
(virus, bacteria, fungi, protozoa) can give rise to HLH. In the study by
Ramachandran, et al. [3], dengue was found to be the leading
organism accounting for 5 among 43 cases of HLH. Veerakul, et al.
[4] also reported a series of 52 pediatric patients with HLH; 15 were
infection associated out of which 3 were caused by dengue. Tan, et al.
[5] also reported a comprehensive report of six cases of dengue
associated HLH in adults. In our series, we had 8 cases of dengue
associated HLH among 358 admitted dengue patients over a time period of
5 months.
Persistence of fever beyond 7-8 days is unusual in
dengue and if it persists, secondary sepsis or dengue associated HLH
should be kept in mind. In our series, all 8 children presented with
fever of more than 7 days along with persistent or progressive
cytopenias, unusual organomegaly with clinical deterioration and sterile
cultures.
Early identification and treatment with dexamethasone,
even a short course of 4 weeks, can give rise to a good outcome.
Contributors: The study was conceptualized by PP
and PPG; both were also involved in patient management. All authors were
involved in drafting the manuscript.
Funding: None; Competing interests: None
stated.
References
1. Loy T, Alberto AD, Perry MC. Familial
erythrophagocytic lymphohistiocytosis. Semin Oncol. 1991;18:34-9.
2. Janka G, Elinder G, Imashuku S, Schneider M,
Henter J. Infection- and malignancy-associated hemophagocytic syndromes:
secondary hemophagocytic lymphohistio-cytosis. Hematol Oncol Clin North
Am. 1998;12:435-44.
3. 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.
4. Veerakul G, Sanpakit K, Tanphaichitr VS,
Mahasandana C, Jirarattanasopa N. Secondary hemophagocytic
lympho-histiocytosis in children: an analysis of etiology and outcome. J
Med Assoc Thai. 2002;85:S530-41.
5. Tan LH, Lum LCS, Omar SFS, Kan FK.
Hemo-phagocytosis in dengue: Comprehensive report of six cases. J Clin
Virol. 2012;55:79-82.
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