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Clinical Brief

Hyper-Reactive Malarial Splenomegaly: Rare Cause of


Pyrexia of Unknown Origin
Sanjay Verma and Anju Aggarwal

Department of Pediatrics, University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi,India.
[Received March 20, 2006; Accepted July 17, 2006]

ABSTRACT
Hyper-reactive malarial splenomegaly (HMS) or Tropical splenomegaly syndrome(TSS), occurs in areas of high
transmission of malaria. These children usually presents with gross splenomegaly and abdominal discomfort, while fever
is not the usual manifestation in majority of them. It is a disease of young adults and rarely reported below 8 years of age.
Here it is reported a three-year-old child who presented as pyrexia of unknown origin with hepatosplenomegaly, diagnosed
as HMS. [Indian J Pediatr 2007; 74 (4) : 409-411] E-mail : aanju@bol.net.in

Key words : Hyper reactive malarial splenomegaly; Pyrexia of unknown origin; Child

Tropical Splenomegaly Syndrome (TSS) or Hyper normal. Reticulocyte count was 2%. Peripheral blood
reactive malarial splenomegaly (HMS), occurs where smear revealed some anisopoikilocytosis with decrease
transmission of malaria is intense. It has been reported in all three cell lines. RBCs were normcytic and
throughout the tropics including India. 1 It usually normochromic. Other hematological parameters
occurs in young and middle aged adults. Fever is not revealed: HbF<1%, HbA 88%, HbA 2 2.6%, which were
the usual presenting feature. We present a 3-yr-old within normal range. Liver function tests revealed a
child with fever of one year's duration. total serum bilirubin of 0.8 mg/dl, ALT 77 IU/L,
alkaline phosphatase 297 IU/L. Total proteins 6.4 g/
dL, albumin 2.1 g/dL, globulin 4.3 g/dL. Malarial
CASE REPORT
parasites were not seen on thick and thin smear
examination. Antigen detection tests for falciparum and
A 3-yr-old boy residing in North India since birth, vivax were negative. Widal test, Montoux test and
presented with intermittent fever for one year. The radiograph of chest were normal. Blood culture and
child complained of dull abdominal pain and urine culture were sterile. Ultrasonography did not
discomfort. Development of the child was normal. He show evidence of ascites, retroperitoneal lymphnodes,
had received one blood transfusion during the illness. subdiaphragmatic or liver abscess or portal
General physical examination revealed moderate hypertension. Bone marrow examination revealed
pallor. Child weighed 10 Kg {below 3rd centile}. Height normal cell lines. Bone marrow iron was normal and
was 85 cm {below 3rd centile}. Abdomen was distended. there was no abnormal pigmentation of macrophages,
Liver was palpable 6 cm below the right costal margin, there was evidence of reticuloendothelial hyperplasia. It
and was firm in consistency and non- tender. The did not reveal any Leishman-donovan(LD) bodies or
spleen was palpable 12 cm below left costal margin and abnormal cells. Antibodies against malarial parasite
was firm in consistency. Examination of other systems were strongly positive (>1:250). Any titre above 1:200 is
was essentially normal. taken as abnormal according to laboratory at National
Insititute of Communicable Diseases. Liver biopsy
Child was investigated for cause of unexplained revealed kupffer cells hyperplasia and lymphocytic
fever, anemia and organomegaly. CBC revealed: Hb infiltrations of the hepatic sinusoids. This was
3.49 g/dL, WBC 2,000/mm 3 , platelets 99,000/mm 3 , consistent with reported findings in HMS. Diagnosis
MCV was 76fl, MCH was 30 pg/dl and RDW was of hyperreactive malarial splenomegaly was
made.Child received a blood transfusion. Child was
given a course of chloroquine. The child was afebrile
Correspondence and Reprint requests : Dr. Anju Aggarwal, Flat after a course of chloroquine and spleen size decreased
No. 3C, Block C2B, Janakpuri, Delhi-110058. by about 1 cm. Child was discharged on weekly

Indian Journal of Pediatrics, Volume 74April, 2007 409


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S. Verma and A. Aggarwal

chloroquine in the dose of 5 mg/Kg. Child did not unusual in these patients. Majority of patients have
come for regular follow- up and had expired after a few associated hepatomegaly; malnutrition and jaundice
months. may be present. Anemia is {normochromic normocytic}
almost always present and associated with
pancytopenia (hypersplenism); and there is increased
DISCUSSION
susceptibility to bacterial infections. Peripheral blood
picture for malarial parasite is usually negative as in
HMS is uncommon in children younger than 8 years, present case.
but it has been reported in a 4-year-old patient. 2 Medical management by antimalarial medicines for
Presentation at 3 years, as in present case, is rare. Early prolonged periods is the mainstay of therapy. Response
presentation may be due to prolonged chronic to therapy is guided by the splenic size and
antigenic stimulation by repeated malarial infections symptomatic improvement. Specific choice of drug is
from early ages in endemic areas. Defining features of dictated by the pattern and prevalence of drug
HMS include: (1)Residence in high prevalence zone for resistance in that geographic area. Chloroquine and
malaria (2)Chronic splenomegaly, often massive, Proguanil appear to be equally effective.2 Chloroquine
usually unexplained by other common conditions phosphate has been used widely for treatment of
(3)Serum IgM elevated to more than 2 SD above the malaria. Dose of chloroquine in HMS is 10 mg/Kg base
local reference mean (4)High malarial antibody titers PO, followed by 5 mg/Kg 6, 24, 48 hours later and then
(5)Hepatic sinusoidal lymphocytosis (6)Clinical and weekly for as long as necessary.7,10 In a study on 5 year
immunological response to long term antimalarial follow-up of TSS patients on chloroquine prophylaxis,
prophylaxis.3-6 Most of the previous studies have used 69% of cases showed a distinct clinical and
combination of 3-4 of the above mentioned criteria for biochemical improvement after chloroquine
diagnosis of HMS. 6,7 In this child living in endemic prophylaxis. 6 Studies have shown that younger
area, the authors found splenomegaly, high malarial patients respond better to chloroquine prophylaxis
antibody titres, liver biopsy suggestive of HMS and then older ones. 11 Recent literature doesnt support the
clinical response to chloroquine treatment. Other role of splenectomy in management of TSS, as fatality
causes of splenomegaly were ruled out. Hence following splenectomy is very high because of
diagnosis of HMS was confirmed. fulminant and overwhelming infections. Splenectomy
Although the exact mechanism of HMS is unknown, is recommended in highly selected patients in whom
evidence suggests that exposure to malaria elicits an there is severe hypersplenism and no response to long
exaggerated stimulation of polyclonal B lymphocytes, term antimalarials. 12
leading to excessive and partially uncontrolled
production of IgM as the initiating event. Defective Hyper-reactive malarial slpenomegaly syndrome
immunoregulatory control of B lymphocytes causes an can present as unexplained fever at a young age
increase in B lymphocytes and a decrease in T specially in tropical countries like India, where malaria
lymphocytes in the peripheral blood. This is is widely endemic. The authors must keep a high index
accompanied by T cell infiltrations of the hepatic and of suspicion to diagnose such cases.
splenic sinusoids and increase in serum cryoglobulin
levels, autoantibody levels and immune complexes. REFERENCES
This leads to anemia, deposition of large immune
complexes in kupffer cells in liver and spleen,
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presenting symptoms of HMS as seen in our child. chloroquine prophylaxis. J Indian Med Assoc 1987; 85: 298
Child had intermittent fever for one year, which is 300.

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Hyper Reactive Malarial Splenomegaly : Rare Cause of Pyrexia of Unknown Origin

8. Piessens WF, Hoffman SL, Wadee AA. Antibody-mediated 10. Raval N, Shah N, Vani SN. Tropical splenomegaly
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