Tropical Splenomegaly Syndrome Research Articles

Splenomegaly in Hmong Refugees

We review asymptomatic splenomegaly in Indochinese refugees and provide recommendations for evaluation of the problem. Prevalence of splenomegaly in newly arrived Indochinese refugees was 2.5%, three times more prevalent in the Hmong than in the non-Hmong refugees. Male Hmong refugees aged 15 to 29 years had the highest prevalence (10%). For the 50 Hmong refugees studied, there was no evidence that their splenomegaly was caused by clonorchiasis, schistosomiasis, tuberculosis, syphillis, lymphoma, tropical splenomegaly syndrome, or clinical malaria. Cases were more likely to have hepatomegaly, hepatitis B surface antigen positivity, and a low mean corpuscular volume than a reference population of Hmong refugees. Malaria antibody titers were elevated in all but one of the 41 cases (98%) tested.

Obscure splenomegaly in the tropics that is not the tropical splenomegaly syndrome.

Le «syndrome de splenomegalie tropicale» est considere comme souvent du a des reponses immunes aberrantes au paludisme. Cette etiologie s'appuie le plus souvent sur: les anticorps antipaludiques, les taux eleves d'IgM seriques, la reponse au traitement antipaludique, la biopsie hepatique montrant une lymphocytose dans les sinusoides. Sur 171 cas de splenomegalie examines, la plupart remplissent 3 ou 4 de ces conditions. Dans les autres cas, une autre etiologie doit etre cherchee

Síndrome de splenomegalia tropical: Presentación del primer caso confirmado en Colombia

The first reported case of tropical splenomegaly syndrome in Colombia is described in a 9-year-old girl who had lived in the Macarena region of the Department of Meta. She had had recurrent P. vivax infection, splenomegaly of 10 cm. below the costal margin, anemia, cryoglobulinemia, increased serum IgM, inmunofluorescent antibody titers to P. falciparum of q:2, 560 and several thick blood smears negative for malaria parasites. Spleen biopsy showed a non-specific reactivity and the external bone marrow aspiration was negative for any pathological changes or parasites. Reduction on both IgM values and splenomegaly and improvement on her clinical condition was seen with no specific antimalarial treatment after a period of observation of three months. Prohylactic treatment with chloroquine, 10 mg/kg of body weight on a weekly basis was established before returning to the endemic area of the Macarena region.

Tropical splenomegaly syndrome (T.S.S.) in a European

A case of tropical splenomegaly syndrome in a 66-year-old white man who had lived in Tanzania for 34 years is described. He had taken anti-malarial prophylaxis continuously and regularly. He had had malaria in 1955 but there was no history of alcoholism or jaundice. He was treated with proguanil hydrochloride and, after return to Tanzania, took paludrine as prophylactic. One year later he had no further complaints, the spleen was no longer palpable and the liver only just palpable.

Esophageal varices in Nairobi, Kenya: a study of 68 cases.

Sixty-eight patients with proven esophageal varices wer studied at Kenyatta National Hospital, Nairobi, Kenya. Of these patients, 29.4% had schistosomal portal hypertension, 22.1% cirrhosis and only 8.8% extrahepatic portal vein occlusion. One quarter of the patients had a normal liver biopsy and extrahepatic portal vein that was demonstrated to be patent. Problems relating to liver biopsy sampling resulting in underdiagnosis of specific causes of esophageal varices such as schistosomiasis are discussed. We argue that many of these patients were likely to be suffering from idiopathic portal hypertension, a condition apparently not previously recognized in Africa. Of this last group, 70.6% had suffered gastrointestinal bleeding, as had 50% of the patients with schistosomiasis. Together these two groups accounted for three-quarters of all patients who had bled. The detection of eggs of Schistosoma mansoni in stool and/or rectal snip correlated well with liver biopsy findings in both a positive and negative sense. Only 18% of patients with negative stools and snips had evidence of schistosomiasis in the liver, and positive stools or snips were found in only 14.6% of patients without schistosomal liver involvement. Of the patients in the study, 50% were of the Kamba tribe, although only 12.9% of all medical admissions to the hospital were Kamba (P less than 0.01). Luo patients were significantly more frequent within the group with schistosomiasis (P less than 0.02). Esophageal varices were attributed to tropical splenomegaly syndrome in only one patient. The implications of our results are discussed and our findings are compared with previous work from East Africa.

7 - Tropical Splenomegaly: Part 1: Tropical Africa

The emergence of tropical splenomegaly syndrome as a distinct entity in tropical medical practice has been briefly described, together with its link with malaria. The clinical and haematological aspects of the syndrome have been reviewed with some emphasis on local experience. No attempt has been made to give details of the immunological aspects, but the mechanisms by which the established immunological features of the disease help to understand the pathogenesis of the syndrome have been highlighted.

Tropical splenomegaly: surgical aspects

At the University College Hospital, Ibadan, Nigeria, in a 22-month period from January 1975 to September 1976, nine splenectomies were electively performed for unruptured spleens. The diagnoses were either tropical splenomegaly syndrome (when no obvious cause was found to be responsible for, or associated with, the splenomegaly) or splenic abscess in various stages of formation. The weights of the spleens removed ranged from 160 g in a 15-year-old girl to 5080 g in a 39-year-old male. One death due to uncontrollable bleeding from the splenic bed occurred. Histology of the spleens after removal showed lymphosarcoma in one, non-specific changes in another, and thickened fibrous capsule, perisplenitis, haemorrhagic infarcts, pockets of chocolate-coloured fluid and inflammatory processes in various stages in the other seven. In the tropics, enlarged spleens with no obvious cause or associated condition are often best treated by splenectomy for histological diagnosis, definitive treatment and the prevention of rupture caused by minor trauma. There are, as yet, no convincing data which show that splenectomy in the tropics causes a significant diminution of immunity to malaria (or other diseases) and it does not, therefore, predispose to the development of cerebral malaria.

Hepatitis B e antigen in the absence of hepatitis B surface antigen.

Hepatitis B e antigen (HBeAg) was detected by agar gel diffusion in the serum of four Ugandan adults (three patients with tropical splenomegaly syndrome and one healthy adult) who did not have detectable hepatitis B surface antigen (HBsAg) by radioimmunoassay. Two of them had antibody to hepatitis B core antigen, and the other two had antibody to HBsAg. Detection of HBeAg by a relatively insensitive immunodiffusion test in the absence of HBsAg detectable by a sensitive radioimmunoassay suggested that production or removal of these two antigens may occur independently.

Massive splenomegaly in Northern Zambia. II. Schistosomal splenomegaly and elevated IgG

Twenty-three patients from Northern Zambia with massive splenomegaly due to chronic schistosomiasis were studied. The mean serum IgG levels were found to be extremely high compared with local blood donors and local patients with Tropical Splenomegaly Syndrome (TSS). Mean serum IgG levels also appear to be higher in Zambians with schistosomal splenomegaly than in those reported in patients from most other areas. Mean IgM levels of patients with schistosomal splenomegaly were higher than those of local blood donors, but not as high as in the TSS. IgG and IgM estimations are sometimes of value in the differential diagnosis of splenomegaly in the tropics.

Hepatitis-B virus determinants in patients with liver disease in Malawi

Sera from 185 patients admitted to hospital in Malawi with acute and chronic liver disease were tested for the following serological markers of hepatitis-B infection (HBV): hepatitis-B surface antigen (HBsAg) and its antibody (anti-HBs), HBe antigen (HBeAg) and its antibody (anti-HBe), and antibody to hepatitis-B core antigen (anti-HBc). Control groups consisted of patients without liver disease, students and blood donors. HBsAg was detected in 73%, 54·8% and 64% of patients with acute hepatitis, cirrhosis and hepatoma, respectively. 16% of patients with tropical splenomegaly syndrome (TSS) and 14·6% of patients with schistosomiasis, 9·5% of patients without liver disease, 1·4% of students and 7·6% of blood donors were HBsAg positive. Unexpectedly, 72·3% of HBsAg-positive specimens appeared to carry both major antigenic determinants ( ady). HBeAg was detected in 15·8% and anti-HBe in 13·1% of patients with acute HBsAg-associated hepatitis, these determinants being detected less frequently than in sera from patients with HBsAg-associated hepatitis in the UK. HBeAg or anti-HBe were rarely detected in patients with hepatoma. With the exception of students and blood donors, over 80% of the Malawian study population had markers of current or previous HBV infection in their sera. Anti-HBc occurred most frequently in patients with hepatitis, cirrhosis and hepatoma, their geometric mean titres (GMTs) being 67·6, 160·1 and 211, respectively. The GMTs of patients with TSS, schistosomiasis and non-hepatic disease were 2·0, 4·6 and 2·3, respectively.

Cold agglutinins and their relationship to malaria

It is well recognized that cold agglutinins frequently occur in high titre in the sera of Melanesian inhabitants of Papua New Guinea. There have been numerous studies of the specificities of these Melanesian cold agglutinins, but few of their pathogensis. They are known to be a common accompaniment of tropical splenomegaly syndrome in New Guineans, but their prevalence in other coastal New Guineans is less well documented, and their relationship to malarial infection has not been clarified. The present study included 332 New Guineans living in the one malarious area, none of whom had received regular suppressive antimalarial therapy prior to this investigation. Cold agglutinins in a titre of greater than 1:32 were rare in young children, but occurred in 6% of older children and in 28% of adults. The corresponding rates after 12 mth of protection against malaria were 2% and 10% respectively. Treatment of acute attacks of malaria only, without regular prophylaxis, produced no such change in a control group. Cold agglutinin titres showed a significant correlation with serum IgG and IgM concentrations, and with titres of antimalarial antibody as measured by indirect immunofluorescence, both in initial values and in subsequent decreases. Values in subjects with tropical splenomegaly syndrome were no higher than in the rest of the community. These data suggest that in Melanesians the production of cold agglutinins forms part of the normal immune response to recurrent malarial infections experienced over many years. The phenomenon does not occur in other races exposed to a similar pattern of malaria, and this may indicate a genetically determined response to a weak antigen released or exposed in parasitized red cells.

Massive splenomegaly in Northern Zambia. I. Analysis of 344 cases

Massive splenomegaly is common in Northern Zambia. Nearly 40% of 344 patients were diagnosed as Tropical Splenomegaly Syndrome of malarial origin. Portal hypertension, lymphoreticular and haemopoietic malignancies, and schistosomiasis accounted for another 46%. The common causes of splenomegaly show differences of frequency within Zambia and also between Zambia and other African countries.

Length of antibiotic therapy in in-patients with primary pneumonias.

Seventy-three subjects with primary pneumonia in a hospital in Northern Nigeria were given antibiotics until they had been afebrile for 24 hours. The average duration of therapy was 2.54 days (about 60 hours), which is considerably shorter than the current recommended practice. Subjects with hepatosplenomegalic schistosomiasis and tropical splenomegaly syndrome required antibiotics for a significantly longer period (3.75 days) than those without either of these conditions. Those with an antigenaemia did not require antibiotics for a significantly longer period than those without an antigenaemia. There were no deaths, no increase in morbidity and in virtually all cases complete resolution of the lung lesion occurred within the expected time. It is suggested that in primary pneumonia it is more rational to stop antibiotics after the patient has been afebrile for 24 hours. This leads to a shorter stay in hospital and to the use of less antibiotic.

Abnormal haemoglobins in the Sudan savanna of Nigeria

Children born in areas hyperendemic for Plasmodium falciparum are protected by maternal antibodies for up to about five months of life, after which they are subject to intense infection until they acquire sufficient immunity--by about five years of age. Children with sickle cell trait (Hb.AS) are at an advantage during these critical years, probably because of preferential phagocytosis of parasitized red cells. This could lead to either (i) early processing of antigen by macrophages and an accelerated immune response, or (ii) less antigenic stimulus and hence lower antibody production. Immunoglobulin (Ig)G and IgM determinations, agar gel diffusion (Ouchterlony) against soluble P. falciparum antigen, the indirect fluorescent antibody (IFA) test using P. falciparum and P. malariae antigens, and the indirect haemagglutination (IHA) test with P. falciparum antigen were performed on sera from a population with different Hb electrophoretic types in the hyperendemic malarial area of Garki, Kano State, Nigeria. Plasma immunoglobulins and antimalarial antibodies rose with age. After the first year of life, lower mean concentrations of immunoglobulins (especially IgM), and lower mean titres of antibodies specific against P. falciparum (Ouchterlony, IHA and less significantly IFA) were present in Hb.AS compared to Hb.AA; these differences increased with age. Antimalarial intervention was followed by a decline of all values and final levels showed little difference between haemoglobin types. It was unlikely that either a relative inability to produce antibody or a more rapid catabolism of immunoglobulins was responsible for the lower levels in sickle cell trait. The observations are more easily explained by the hypothesis that Hb.AS persons have less antigenic stimulus due to the early removal of parasitized sickled cells by macrophages, which then degrade the antigens. The antibody difference between Hb.AA and Hb.AS increased throughout life, suggesting that this process remained a feature of sickle cell trait even after parasite frequencies and densities were similar in the two Hb groups. These observations have implications in the aetiology of tropical splenomegaly syndrome, which is rarely seen in sickle cell trait subjects. Mean IgG and IgM were slightly higher in Hb.AS than Hb.AA infants, the difference for IgG achieving significance. This suggested that during infancy early phagocytosis of parasitized cells had led to enhanced processing of antigen and hence an earlier immune response in Hb.AS, but this was unlikely to be a major factor in survival. IFA titres against P. malariae were slightly but not significantly lower in Hb.AS, possibly as a result of cross-reaction with P. falciparum antibody or of a slight degree of protection against P. malariae.

Malarial antibodies in tropical splenomegaly syndrome in Papua New Guinea

Levels of species and class-specific malarial antibody were studied in 249 New Guineans with tropical splenomegaly syndrome (TSS) and in 87 control subjects living in the same area. Titres of IgG and IgM antibody to Plasmodium falciparum, P. vivax and P. malariae were estimated by indirect immunofluorescence. Both IgG and IgM antibody levels were higher in subjects with TSS than in controls; IgM titres were highest in those with the greatest splenic enlargement. Responses to all three species were comparable. It is concluded that there is no evidence from this study to incriminate any one species of malaria parasite in the production of tropical splenomegaly syndrome.

A SUPPRESSOR T-CELL DEFECT IN TROPICAL SPLENOMEGALY SYNDROME

Serum-IgM is always abnormally high in tropical splenomegaly syndrome. It is postulated that patients with this disease have an abnormal immunoglobulin response to malaria because they lack effective T suppressor cells. This defect may be genetically determined, thus explaining the tribal and familial aggregation of the disease. Hypermacroglobulinæmia is associated with the formation of large amounts of high-molecular-weight immune complexes. These complexes are important in the pathogenesis of the clinical features of the syndrome.

Metabolism of IgM in the tropical splenomegaly syndrome

IgM metabolism was studied in five patients with the tropical splenomegaly syndrome and in five controls. The half-life and turnover time of I 125 IgM was similar in the two groups. We conclude that the raised serum IgM levels found in patients with the tropical splenomegaly syndrome are not due to decreased IgM catabolism but are due to increased IgM production.

Fulminant tropical splenomegaly syndrome.

Eight patients with the tropical splenomegaly syndrome who presented with an acute febrile illness and severe anaemia due to haemolysis are described. None was pregnant. All were treated with anti-malarial drugs, four received steroids, and another two steroids and other cytotoxic drugs. Two patients died from causes which were, in part, attributed to this complication of the tropical splenomegaly syndrome. This mode of presentation is seldom documented in this condition. The management and differential diagnosis are discussed.

THE TROPICAL SPLENOMEGALY SYNDROME

Medical Journal of AustraliaVolume 2, Issue 16 p. 625-626 COMMENT THE TROPICAL SPLENOMEGALY SYNDROME First published: October 1975 https://doi.org/10.5694/j.1326-5377.1975.tb106153.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume2, Issue16October 1975Pages 625-626 RelatedInformation

The rôle of plasma proteins in chronic expansion of plasma volume in tropical splenomegaly syndrome: III. The interrelationships of albumin, immunoglobulins and plasma volume

The effect of alterations in serum protein levels on plasma volume in tropical splenomegaly syndrome has been investigated by determining plasma volume, serum albumin, globulin and immunoglobulin levels in 64 adult New Guineans suffering from this disease. Plasma volumes ranged from 51.2 to 129 ml./kg. Significant positive correlations were found between plasma volume and intravascular pools of albumin, IgG and IgM. Multiple regression analysis demonstrated that 70% of the variance in plasma volume in this series was attributable to increases in these three pools, IgM and IgG accounting for 42% of the total and albumin for 28%. It is probable that the unexplained 30% of plasma volume expansion arises through a splenic arterio-venous shunt effect. It is suggested that initially in tropical splenomegaly syndrome the splenic shunt and immunoglobulin overproduction combine to produce expansion of plasma volume and a fall in intravascular colloid osmotic pressure; that the latter provides the stimulus to increased albumin synthesis, and that this further aggravates the plasma volume expansion and the consequent dilutional anaemia commonly seen in this disease.