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Indian Pediatr 2012;49: 993 |
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Hemophagocytic Lymphohistiocytosis as
Presenting Feature of Lupus
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Swati Kalra and Anju Aggarwal
Department of Pediatrics, University College of Medical
Sciences,
and Guru Tegh Bahadur Hospital, New Delhi, India.
Email: [email protected]
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Hemophagocytic lymphohistiocytosis (HLH) is a multisystem
disorder characterized by dysregulation of the immune system
with hypercytokinemia and hyperinflammation. It can be
primary or secondary [1]. Rheumatic diseases rarely cause
secondary HLH. Systemic lupus erythromatosus (SLE) as a
cause of HLH is rare [2-4].
A 7-year-old boy presented with fever of
one month duration. On examination, he was febrile, liver
and spleen were palpable 4 cms below costal margin. Initial
investigations revealed a hemoglobin of 8.2 g/dL,
TLC-1800/mm 3,
Platelet-42,000/mm3,
MCV-94.6 fl, MCH-25.3 pg, MCHC-26.7g/dL. His peripheral
smear revealed pancytopenia, macrocytes and microcytes.
Serum widal titers were below 1:80. Blood culture and urine
culture were sterile. Total protein was 4.8 g/dL,
Albumin:globulin ratio was 0.5:1, Total bilirubin-2.7 mg/dL
and were liver elevated enzymes (SGOT-180U/L, SGPT-513 U/L
and ALP-570 U/L). Urea and creatinine were within normal
range. Mantoux was non-reactive. He developed nasal bleeding
and oral ulcers. Fever persisted inspite of treatment with
ceftriaxone for seven days. Ultrasound of chest and abdomen
revealed mild pericardial effusion, pleural effusion and
mild ascites. Prothrombin time and activated prothrombin
time were deranged, platelet counts were below 50,000/mm3
and direct Coomb’s test was negative. He received 4 unit of
platelets and 2 unit of red cell concentrate transfusion
within 10 days because of recurrent epistaxis, fall in
hemoglobin and platelet count. Bone marrow aspiration
revealed features suggestive of hemophagocytic syndrome
(increase in histiocytes with hemo-phagocytosis).
Triglyceride levels were 202 mg/dL. The diagnostic criteria
of HLH syndrome were fulfilled [1]. In view of multisystem
involvement with presence of pancytopenia, oral ulcers and
serositis, diagnosis of SLE was considered. Antinuclear
antibodies by ELISA method were positive. Anti-double
stranded DNA levels were 169.9(>55 IU/mL significant). Child
was diagnosed as having SLE as per the diagnostic criteria
of SLE [5]. Renal biopsy did not reveal any abnormality.
Child was given steroids (prednisolone 2 mg/kg/day). There
was improvement in the clinical condition of the child,
pleural effusion and ascites subsided. On follow up child
did not have fever, bleeding manifestations, anemia or
serositis. His hemoglobin was 11g/dL, TLC-7800/mm3,
and platelets-1.2 lakh/mm3.
There are few case reports where SLE has
manifested as HLH in a male child. Rajam, et al. [2]
described two cases of HLH in rheumatic diseases in
childhood. An adolescent girl with systemic onset juvenile
idiopathic arthritis presented like severe sepsis and shock.
The other patient presented with cardiac tamponade and was
later diagnosed as SLE. Taki, et al. [3] reported a
21-year-old male diagnosed as SLE and HLH simultaneously. He
was treated successfully with high-dose prednisolone. Yeap,
et al. [4] described 14-year-old girl in whom HLH was
the initial presentation of SLE. HLH as presenting feature
of SLE in a male child is rare, as in our case.
References
1. Freeman HR, Ramanan AV. Review of
hemophagocytic lymphohistiocytosis. Arch Dis Child.
2011;96:688-93.
2. Rajam L, Prasad V, Yatheesha BL.
Reactive hemophagocytic syndrome. Indian J Pediatr. 2008;
75:1261-3.
3. Taki H, Shinoda K, Hounoki H, Ogawa R,
Hayashi R, Sugiyama E, et al. Presenting
manifestations of hemophagocytic syndrome in a male patient
with systemic lupus erythematosus. Rheumatol Int.
2010;30:387-8.
4. Yeap ST, Sheen JM, Kuo HC, Hwang KP,
Yang KD, Yu HR. Macrophage activation syndrome as initial
presentation of systemic lupus erythematosus. Pediatr
Neonatol. 2008;49:39-42.
5. Tan EM, Cohen AS, Fries JE, Masi AT, McShane DJ,
Rothfield NF, et al. The 1982 revised criteria for
the classification of systemic lupus erythematosus.
Arthritis Rheum. 1982;25:1271-7.
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