Recent studies of hyperreactive malarious splenomegaly (tropical splenomegaly syndrome) in Papua New Guinea.
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AIM: The purpose of this study was to determine the prevalence of splenomegaly on chest radiographs of HIV-infected outpatients and to correlate with CD4 cell counts, opportunistic conditions, liver disease and the presence of intrathoracic disease on chest radiographs. METHODS: We reviewed sequential chest radiographs of 200 HIV Clinic outpatients at the Jacobi Medical Center, Bronx, New York, mixed with chest radiographs of 137 outpatients of unknown HIV status (control group) for the presence of splenomegaly and intrathoracic disease. Chest radiographic assessment of splenomegaly was correlated with computed tomography (CT) or ultrasound (US) in 90 out of 337 patients (27%). Clinical charts of all HIV-infected patients were reviewed for CD4 cell count, liver disease and opportunistic conditions including those associated with splenomegaly. The HIV-infected patients were divided into four groups by ascending CD4 cell count: Group 1 (0-50 cells/mm3), Group 2 (51-200 cells/mm3), Group 3 (201-500 cells/mm3) and Group 4 (>500 cells/ mm3). There were 118 men and 82 women with a mean age of 40 (range 20-60) years. Mean CD4 was 180 (range 2-1108) cells/mm3. We also reviewed all autopsies (n = 239) performed on HIV-infected patients between 1983 and 1995 at our institution to correlate splenic size with splenic pathology in that population. RESULTS: Splenomegaly was present on chest radiographs in 82 (41%) HIV-infected patients including: 36/84 (43%) Group 1, 23/49 (47%) Group 2, 18/46 (39%) Group 3, and 5/21 (24%) Group 4 (P = NS). Splenomegaly was present in 30/97 (31%) patients with no evidence of liver disease or opportunistic conditions known to be associated with splenomegaly. Forty-nine HIV-infected patients had 63 opportunistic conditions known to be associated with splenomegaly (mycobacterial and fungal infections, Kaposi sarcoma and lymphoma), half of whom had splenomegaly. Splenomegaly was present on chest radiographs in 18/137 (13%) controls. The presence or absence of splenomegaly on CT or US agreed with chest radiography in 89%. Among the autopsied patients, 135/239 (56%) had splenomegaly (splenic weight > or = 240 g). No specific pathogen was present in 93/135 (69%) enlarged spleens. In contrast, one or more opportunistic conditions were present in 26/104 (25%) normal weight spleens. CONCLUSION: In conclusion, splenomegaly is common in HIV-infected patients and was present in 41% of this series. Splenomegaly may be seen in HIV-infected patients without associated opportunistic conditions or liver disease and in the absence of specific splenic pathology. Chest radiography plays an important role in detecting splenomegaly and may lead to earlier diagnosis of HIV infection.
Chronic splenomegaly in 131 Kenyan patients was investigated at Kenyatta National Hospital, Nairobi. Patients were allocated to diagnostic groups on the basis of clinical, haematological, parasitological, histological, radiological and endoscopic data. The major diagnostic groups were hyper-reactive malarial splenomegaly, our preferred name for tropical splenomegaly syndrome, (31%), hepatosplenic schistosomiasis (18%), visceral leishmaniasis (5%) and "indeterminate splenomegaly", where no diagnosis could be reached (12%). Another 20% of patients were suffering from various non-schistosomal forms of portal hypertension. A number of specific and rarer causes accounted for the rest of the cases. The tribal and geographical distribution of patients with chronic splenomegaly was compared with the pattern of general medical admissions. Splenomegaly was more frequent than expected in Kamba and Luo patients. Hyper-reactive malarial splenomegaly and hepatosplenic schistosomiasis were common in both groups, whereas visceral leishmaniasis was almost restricted to the Kamba and indeterminate splenomegaly was especially prevalent in the Luo. Malarial antibody and immunoglobulin levels differed significantly between the various diagnostic categories of patients and controls. Malarial serology can be diagnostically useful for chronic splenomegaly, provided results are interpreted in their geographical context.
Splenomegaly and parasitaemia were correlated in 2891 children examined in outpatient clinics during 13 consecutive months in 4 Punjabi villages. The average monthly splenomegaly rate varied from 0.05-0.13 before the monsoon malaria transmission season to 0.18-0.27 during and after this season. Moderate splenic enlargement reached a peak during the malaria transmission season, while the highest proportion of very enlarged spleens occurred towards the end of, and after, the transmission season. Children with splenomegaly were 3 times more likely to have Plasmodium falciparum and 1.5 times more likely to have P. vivax parasitaemias than were children without palpable spleens. The larger the spleen the more likely a P. falciparum infection, whereas P. vivax was more commonly associated with minimal spleen enlargement. Although the probability of a child with splenomegaly having a malaria parasitaemia was highest (0.58-0.72) during and immediately following periods of malaria transmission, the odds ratio of malaria infections among those with splenomegaly to those without was at its lowest (1.41-2.11) during those months. Mean P. falciparum parasitaemias were significantly higher in infected children with moderately enlarged spleens than in infected children with nonpalpable spleens or in those with minimal or extensive splenomegaly. These results are compatible with splenomegaly being a result of both the malaria infection and the immune response. Early in infection many children had parasitaemia without splenomegaly; after the parasitaemia had cleared splenomegaly often persisted.
The haemolytic process in hepatosplenic schistosomiasis is often regarded as a result of splenomegaly that can be increased by spontaneous or surgical shunts. The importance of splenomegaly and/or shunt on the etiology of haemolysis was studied in a group of 74 patients with hepatosplenic schistosomiasis and portal hypertension. They were divided into 4 groups: Group I (22 cases) - pre-operative; Group II (15 cases) - classic splenorenal shunt; Group III (22 cases) - selective portal decompression and Group IV (15 cases) - azygoportal disconnection with splenectomy. The first group is characterized by the presence of splenomegaly and the absence of shunt; the second by the presence of shunt and absence of splenomegaly; the third, by the presence of both splenomegaly and shunt and the last by absence of both, shunt and splenomegaly. In all cases, the levels of sensitive markers of haemolysis, such as haptoglobin and hemopexin as well as non-conjugated and total bilirubins were determined. Biochemical and coagulation parameters, used to evaluate the hepatic function, were also studied. The data was initially analysed according to a factorial experiment of 2 and standard least squares techniques were used to evaluate the effects of splenomegaly, shunt and their interaction. The results showed that the shunts always provoked haemolysis, even in the absence of splenomegaly, but the presence of both, splenomegaly and shunt, always increased the process of haemolysis.
This study aimed to investigate the prevalence of palpable splenomegaly in children with haemoglobin SC (Hb SC) disease, and to determine the haematological and clinical manifestations of splenomegaly in this patient population. We performed a retrospective chart review of 100 patients with Hb SC over 2 years of age followed by the Duke University Paediatric Sickle Cell Program with serial physical examinations and laboratory measurements. Palpable splenomegaly was present in 34% of patients and was more common in males (P = 0.029). Children with splenomegaly had a significantly lower average haemoglobin concentration (10.3 vs. 10.8 g/dl, P = 0.011) and lower platelet count (237 vs. 314 x 109/l, P < 0.001) than those without splenomegaly. Children with measurements both before and after the onset of splenomegaly had a significant decrease in the platelet count (279 vs. 216 x 109/l, P < 0.001) and white blood cell count (9.1 vs. 7.9 x 109/l, P = 0.04) after splenomegaly was identified. Clinical complications included acute splenic sequestration in 12% of children (median age 5.4 years), and hypersplenism with chronic thrombocytopenia in another 10% of patients (median age 10.6 years). Splenomegaly is a common physical finding in children with Hb SC disease and is often associated with mild cytopenias. Clinical complications of splenomegaly include acute splenic sequestration in younger patients and hypersplenism with chronic thrombocytopenia in older children.
Thirty seven patients with sarcoidosis were examined using ultrasound (US) to determine the size of the spleen. A Spleen Index (SI) was employed to evaluate splenomegaly and the SI was calculated using long (a) and short (b) dimensions on the sectional splenotomogram (SI = a x b). In 21 (57%) of these patients the spleen was judged ultrasonographically to be enlarged (SI 30), but in only 3 was it palpable. The clinical records of patients with and without splenomegaly detected by US were compared. There were no differences between patients with or without splenomegaly in hematologic findings (peripheral blood and bone marrow) or blood chemistry; furthermore no patients with hypersplenism were seen. In immunological parameters, the serum immunosuppressive acid protein level was significantly (p less than 0.05) higher in patients with splenomegaly than in those without splenomegaly; however, there were no differences in serum angiotenins converting enzyme activity, serum lysozyme level, PPD skin test or bronchoalveolar lavage fluid analysis. The patients with splenomegaly had significantly higher evidence of increased uptake of 67-Gallium in lung fields and positive lung infiltrates in chest X-ray than those without splenomegaly (p less than 0.01, p less than 0.05). These data suggest that ultrasound is a promising diagnostic tool for the assessment of the size of the spleen and is useful to detect disease activity and extent of disease in sarcoidosis. Patients with sarcoidosis who had splenomegaly had more disseminated disease, especially pulmonary parenchymal disease, than did those without splenomegaly.
HYPOTHESIS: Laparoscopic splenectomy (LS) is the procedure of choice for elective splenectomy. Splenomegaly may preclude safe mobilization and hilar control using conventional laparoscopic techniques. Hand-assisted LS (HALS) may offer the same benefits of minimally invasive surgery for splenomegaly while allowing safe manipulation and splenic dissection. DESIGN: A retrospective review of patients with splenomegaly undergoing conventional LS or HALS was performed. SETTING: Tertiary care referral center. PATIENTS: Hand-assisted LS was performed at the start of the operation for patients with splenomegaly; splenomegaly was determined by palpation of the splenic tip extending to the midline or the iliac crest, or by a craniocaudal splenic length of greater than 22 cm. Splenomegaly was defined as a splenic weight of greater than 700 g after morcellation. MAIN OUTCOME MEASURES: Patient demographic characteristics, operative indications, splenic weight after morcellation, morbidity, mortality, and clinical outcomes were evaluated. RESULTS: Forty-five patients with splenomegaly were identified: 31 underwent standard LS and 14 underwent HALS. The HALS group had significantly larger spleens than the conventional LS group (mean weight, 1516 vs 1031 g; P =.02). Mean operative time (177 vs 186 minutes; P =.89), estimated blood loss (602 vs 376 mL; P =.17), and length of hospital stay (5.4 vs 4.2 days; P =.24) and complication rates (5 [36%] of 14 vs 5 [16%] of 31; P =.70) were similar between the HALS and the standard LS groups. No perioperative mortality occurred. CONCLUSIONS: Hand-assisted LS is a safe and efficacious procedure for these extremely difficult cases. Hand-assisted LS provides the benefits of a minimally invasive approach in cases of splenomegaly.
The morphometric analysis was carried out for 150 cases splenomegaly pathological sections (1010 sections) after being stained with histochemical, immunohistochemical, hematoxylin and eosin methods, summarizing that the proliferation of three types of fibers, the number and distribution of T, B lymphocytes and macrophages, the changes of sections stained with HE, the ultrastructure of splenomegaly under electron microscope would be the criteria for partially preserving splenomegaly. The data of morphometry was treated by IBM/PC computer. The research was aimed to establish a pathological indication for preserving partial splenomegaly clinically. The study demonstrated that the pathological classification, based on the fibrosis degree of splenomegaly, may objectively reflect the changes of immunological function of splenomegaly, by which it was also revealed that the immunological function in pathological class II and III was markedly lowered. Because the proportion of class I splenomegaly remained only in 31% of good function, there are less splenomegaly worth preserving.
Splenomegaly was studied retrospectively at the University of California, San Francisco, School of Medicine, on all patients (N = 2,056) for the years 1913 to 1962. The patients were classified into several diagnostic groups, and these groups were tested for statistical significance (chi(2)) with many clinical and laboratory variables to determine their predictive value. Hematologic disorders were associated with 57% of cases of splenomegaly and 81% of cases of massive splenomegaly. Among patients with splenomegaly, 19% had infectious diseases, 11% had hepatic diseases, and 9% had congestive or inflammatory disorders. The residual 4% were considered to have primary splenic disorders or a disorder of unknown cause. The commonest diseases associated with splenomegaly were hematologic (acute and chronic leukemias), infectious (malaria, endocarditis, and tuberculosis), hepatic (chronic liver disease), congestive (congestive heart failure), inflammatory (thyrotoxicosis), and other (cancers not metastatic to the spleen). The diseases most frequently associated with massive splenomegaly were the chronic leukemias. The disease with the highest incidence of massive splenomegaly was myelofibrosis (23 of 29 patients, 78%). Splenectomy was performed in 154 patients (7%), primarily for hematologic amelioration and hepatic hypersplenism. Hematologic diseases showed significant associations with lymphadenopathy, generalized lymphadenopathy, massive splenomegaly, and cytoses (P .001) and with progressive splenic enlargement (P < .02). Infectious diseases showed significant association with fever, and hepatic diseases showed significant association with abnormal results of liver function tests (P < .001). The findings of this retrospective study should be validated prospectively.
Hyperreactive malarial splenomegaly (HMS), a common cause of massive splenomegaly in malaria-endemic regions, is defined as persistent splenomegaly without demonstrable underlying disease. Previous studies have found HMS more frequently in certain tribes in Papua New Guinea, Uganda and Nigeria, with strong familial associations in Uganda and Papua New Guinea. This case-control study aimed to determine the extent of familial association of splenomegaly and the pattern of segregation of the condition in families in Ghana. It involved 22 HMS cases with 99 relatives, and 15 population controls of similar socio-economic background with 51 relatives. The pedigree of each family was recorded. Clinical and laboratory data were collected on all participants, including the presence and degree of splenomegaly. Relatives with splenomegaly were identified for 27% of HMS cases and for 6.7% of population controls (P=0.04). There were significant differences in the IgM levels, which were higher (P=0.005), and the haemoglobin levels, which were lower (P=0.009), in cases compared with controls. In Ghana, relatives of HMS cases are more likely to have splenomegaly than population controls, but with no obvious pattern of Mendelian segregation. HMS aetiology in Ghana is likely to be complex, involving multiple genetic and environmental factors.
AIM AND METHODS: This prospective study was carried on 20 patients (10 with liver cirrhosis and 10 with myelo-lymphoproliferative disorders), consecutively admitted to our ward for splenomegaly and thrombocytopenia, with the aim of evaluating the ability of Duplex-Doppler ultrasonography (DDUS) to discriminate between congestive splenomegaly and enlarged spleen caused by haematological disorders. RESULTS: Comparing the clinical/laboratory and DDUS findings for the two groups, it emerged that maximum-portal flow velocity (PFV) values revealed the most statistically significant differences: 17.31 SD 2.48 vs. 28.27 SD 3.53 (cm/s, P < 0.001). Discriminant analysis showed that max-PFV is the variable which by itself maximizes the separation between the two groups (F = 71.56; P < 0.0001). The patients with congestive splenomegaly exhibited lower max-PFV than the controls (17.31 SD 2.48 vs. 26.29 SD 2.38 cm/s, P < 0.001), unlike those with haematological diseases, whose max-PFV values were greater, albeit not significantly so (28.27 SD 3.53 vs. 26.29 SD 2.38 cm/s, P = 0.161). CONCLUSIONS: DDUS assessment of portal haemodynamics thus proved useful in the differential diagnosis of splenomegaly in asymptomatic patients since it distinguishes rapidly and non-invasively between congestive and haemotological splenomegaly. A lower-than-normal max-PFV value indicates congestive splenomegaly; a highish value, on the other hand, suggests a splenomegaly of haematological origin.
BACKGROUND: The causes and diagnosis of massive tropical splenomegaly are not well studied, especially with modern investigative methods. We aimed to identify features that would help local clinicians differentiate between the underlying conditions. METHODS: We collected prospective clinical and laboratory data on 221 Ghanaian patients with spleen size of at least 10 cm. We identified conditions associated with massive splenomegaly with molecular and immunological investigations as well as routine tests. Patients were assigned to diagnostic categories on the basis of these test results and predetermined criteria. FINDINGS: Hyper-reactive malarial splenomegaly (HMS; 91 patients [41%]) and B-lymphoproliferative disorders (48 [22%]) were the most common disorders associated with massive splenomegaly. Of the remaining patients, 32 (14%) had haematological disorders, and in 50 (23%) we could not identify the cause of splenomegaly. Male sex predominated in all diagnostic groups except HMS and tropical splenic lymphoma. Age less than 40 years and absolute lymphocyte count (less than 10 x 10(9)/L) were the only useful and widely available discriminators for distinguishing patients with HMS from those with lymphoproliferative disorders. INTERPRETATION: B-lymphoproliferative disorders are a previously unrecognised cause of massive tropical splenomegaly. This finding has major implications for management of massive splenomegaly. Diagnosis of the less common causes of this disorder is usually straightforward, but differentiating between B-lympho proliferative disorders and HMS can be difficult. HMS is associated with younger age, a higher proportion of women, and lower absolute lymphocyte counts than lympho proliferative disorders.
It is generally felt that increments in platelet counts following platelet transfusions to patients with splenomegaly are severely reduced as a result of splenic sequestration. The results of 631 random-donor platelet transfusions administered to 66 thrombocytopenic patients with palpable splenomegaly were analyzed. Increasing splenomegaly had a significant effect on corrected count increments (CCIs), with the greatest deterioration occurring in patients whose spleens were palpable greater than 2.0 cm below the left costal margin. A similar trend was noted when the percentage of transfusions with satisfactory CCIs was compared, although it should be noted that 42 percent of the transfusions produced CCIs greater than 7500, and a large proportion produced clinically helpful absolute increments. Splenomegaly also had an effect on platelet survival: in patients with CCIs greater than 7500, the mean ratio of 24-hour CCIs to 1-hour CCIs was 0.29 to 0.47 in patients with various degrees of splenomegaly, as compared to an expected value of 0.6 to 0.7. Sixteen patients, most with spleens palpable less than 5.0 cm below the left costal margin, consistently had CCIs greater than 7500. A significant fraction of patients with splenomegaly can benefit from platelet transfusion, and thus splenomegaly should not preclude intensive therapeutic approaches.