compared with a rate of 6–20% in cerebral malaria. Data on the prevalence of HMS is scant, but in areas with intense transmission of malaria. malarial splenomegaly. SIR—Hyper-reactive malarial splenomegaly (HMS) is a form of severe malaria, with a mortality rate that exceeds 50%,1,2 compared with . ABSTRACT. Hyper-reactive malarial splenomegaly (HMS) or Tropical splenomegaly syndrome(TSS), occurs in areas of high transmission of.
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We present a series of seven individuals who were referred to our clinics with possible HMSS.
Tropical splenomegaly syndrome – Wikipedia
Chronic malaria was demonstrated in those successfully treated but not in those who failed to respond to therapy. This observation suggests that the newer molecular malaria assays have a role to play in the identification of individuals who are likely to respond to treatment for HMSS in non-endemic regions. Hyperreactive malarial splenomegaly syndrome HMSSpreviously tropical splenomegaly syndrome, is a poorly understood condition thought to represent an exaggerated immune response to recurrent or persistent malarial infection.
Published criteria for the diagnosis of HMSS are based on 1 the exclusion of other causes of splenomegaly; 2 immunity to malaria, defined as a strongly positive antibody test; 3 splenomegaly of at least 10 cm; 4 a serum concentration of IgM at least two standard deviations above normal; and 5 a clinical and immunological response to malaria prophylaxis.
A year-old pregnant woman from Sierra Leone was admitted to hospital with shortness of breath and fatigue. She had migrated to the United Kingdom 4 years before.
Physical examination revealed gross splenomegaly. On two occasions, blood film microscopy showed only anemia. However, a solitary trophozoite of Plasmodium falciparum was noted on a third film, performed 1 month after her initial presentation.
Subsequent testing revealed a raised serum total IgM and strongly positive malaria serology. She was treated with a 3-day course of oral quinine mg three times daily and subsequently started on chloroquine mg once weekly, which she took for a month. She developed hemolytic anemia 2 months after stopping chloroquine after further travel to Sierra Leonebut blood films and PCR were negative for malaria. Chloroquine was restarted but changed to proguanil mg once daily OD after 3 weeks. Her splenomegaly and anemia completely resolved within 2 months.
A year-old woman from Sierra Leone, who had arrived in the United Kingdom 6 months before, was referred with abdominal pain. Ultrasound scanning of her abdomen showed 15 cm splenomegaly and increased periportal liver echogenicity, suggesting periportal fibrosis. Schistosomal serology was positive and ova of Schistosoma mansoni were seen on stool microscopy.
Histologic examination of liver biopsy showed pipestem fibrosis. Five years later, she returned to clinic complaining that her abdominal pain had never improved and had recently become more severe. Schistosomal serology was now negative. Blood film microscopy was negative for malaria but serology was strongly positive. Review of the initial histology revealed hemozoin pigment in Kupffer cells.
This finding raised the possibility of chronic malaria, hemozoin is only occasionally seen in chronic schistosomiasis. She was commenced on chloroquine mg weekly and proguanil mg OD.
A repeat ultra sound scan 2 months later confirmed complete resolution of the splenomegaly. A year-old Somalian woman, resident in the United Kingdom for 6 months, presented with abdominal pain and fever. Gross splenomegaly was noted on examination. Malaria serology was strongly positive and IgM spplenomegaly.
Multiple blood films were negative for malaria, but PCR for P. She was treated with quinine for 1 week and was subsequently given 6 months of choloroquine mg weekly and proguanil mg dailywith complete resolution of her splenomegaly. A year-old Nigerian man presented with lethargy and abdominal pain.
He had not traveled to a malarial area in the preceding 7 years. Malarial serology was positive and serum IgM was 7. No malarial parasites were seen on examination of a blood film but a malarial antigen test BinaxNow; Alere was positive. He was treated with a short course of mefloquine followed by 6 months of chloroquine and proguanil, after which his clinical and hematological abnormalities were resolved.
Five years later, his symptoms returned. On this occasion, his splenomegaly did not respond to antimalarial therapy. He subsequently died of a widespread anaplastic malignancy of uncertain origin. A year-old woman from Cote d’Ivoire was referred for investigation of splenomegaly and pancytopenia. Malarial serology was positive but serum IgM was not raised. Microscopy and PCR of whole blood for malaria species was negative on several occasions.
She received 4 months of weekly chloroquine mg, but her splenomegaly and cell counts did not improve. No evidence of chronic malaria. Cause of splenomegaly uncertain. A year-old man was referred to a hospital for investigation of splenomegaly and pancytopenia. He was born in Sierra Leone and had migrated to the United Kingdom 8 years before. Blood tests on presentation showed anemia, thrombocytopenia, and leucopenia.
Further testing showed a high antimalarial antibody titer and raised total IgM. Malaria PCR was negative. After 8 months of treatment with chloroquine, his splenomegaly was unchanged. He was then prescribed a prolonged course of atovaquone—proguanil one tablet ODwith no improvement. He remains under review. A year-old man, who had recently migrated to the United Kingdom from Sierra Leone, was referred to a hospital with abdominal spleno,egaly.
He had experienced febrile episodes with occasional jaundice every 2—3 months for many years and these had been treated in Sierra Leone with empirical antibiotics and antimalarial therapy. Examination showed gross 25 cm splenomegaly. He was diagnosed with hepatitis B and D, liver cirrhosis, and schistosomiasis on the basis of positive serological tests and biopsy. The degree of splenomegaly was felt to be incompatible with liver cirrhosis and maalrial diagnosis of HMSS was considered.
Antimalaria antibody titers were found to be strongly positive and hyperfeactive IgM was raised. He was treated with proguanil mg daily and chloroquine mg twice weekly for a year, followed by atovaquone—proguanil for over 3 years, again with no improvement. Splenomegaly likely related to cirrhosis. This series is the largest described in a non-endemic setting, and the cases illustrate some of the challenges splenomebaly the diagnosis and management of such a poorly understood condition.
All were treated with prolonged courses of antimalarial drugs, as they would have been in their countries of origin. The choice of drug may have been guided by the suggestion from the early s that chloroquine has an immunological effect on HMSS in addition to its antimalarial properties.
Four of the seven cases hyperreacctive we reported responded to malarila therapy. It is notable that each of these individuals had evidence of active low-grade malarial infection. The diagnostic criteria for HMSS were last amended inbefore the advent of molecular tests for malaria. The management of cases of splenomegaly that do not respond to antimalarial therapy represents a particular challenge. Splenomegalh surgical risks of splenectomy are often considered to be unacceptable, which leaves these patients at risk of bleeding and traumatic splenic rupture.
The development of lymphoma also appears to be a significant risk in HMSS. National Center for Biotechnology InformationU. Am J Trop Med Hyg. Author information Article notes Copyright and License information Disclaimer. Received Apr 15; Accepted May mallarial This article has been cited by other articles in PMC.
Introduction Hyperreactive malarial splenomegaly syndrome HMSSpreviously tropical splenomegaly syndrome, is a poorly understood condition thought to represent an exaggerated immune response to recurrent or persistent malarial infection.
Table eplenomegaly Summary of patient characteristics and clinical features. Open in a separate window. Cutoff was mean of the negative controls plus 0. We examined fields in a thick film. Chronic malaria with HMSS and hemolysis; possible reinfection. Chronic malaria with HMSS undiagnosed on initial presentation. Chronic malaria with HMSS. Discussion This series is the largest described in a non-endemic setting, and the cases illustrate some of the challenges in the diagnosis and management of such splemomegaly poorly understood condition.
Hyperreactive Malarial Splenomegaly Syndrome—Can the Diagnostic Criteria Be Improved?
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The hyper-reactive malarial splenomegaly: a systematic review of the literature
Validation of PCR for detection and characterization of parasitaemia in massive splenomegaly attributed clinically to malaria infection. Diagn Microbiol Infect Dis. Reduction of spleen size in a child with hyperreactive malarious splenomegaly HMS treated outside the Brazilian endemic area of malaria with only one course of quinine.