Abstract:
Background: Hyper-reactive malarial splenomegaly (HMS) is a syndrome of massive splenomegaly occurring in a malarious region. There is limited information about the prevalence in Tanzania overall and how these patients present clinically.
Aim: To assess the magnitude of HMS among patients with massive splenomegaly at Muhimbili National hospital, Tanzania
Participants: All patients with massive splenomegaly aged 15 years and above who were seen at MNH for 3 months period, from January-March 2017
Study design: Descriptive cross sectional study
Methods: Consenting participants were recruited from the EMD, cold OPD and outpatient clinics of MNH, A structured questionnaire was used to obtain social demographic particulars; the same also recorded symptoms associated with massive splenomegaly, history of recurrent malaria, history of hepatitis, alcohol abuse, exposure to schistosomiasis, family history of hemoglobinopathies, signs and symptoms of portal hypertension. On physical examination pallor, jaundice, lymphadenopathy, spleen and liver sizes were recorded.
After exclusion of all other causes of massive splenomegaly, i.e. ≥ 10cm below left coastal margin, a diagnosis of HMS was made based on a patient coming from a malaria endemic country, and having a, raised serum IgM.
Results: in a total of 99 study participants, 5 were found to have HMS: 3 of these were females. The median age of HMS patients was 35 years. With respect to common complaints on presentation, all 5 patients had abdominal distension, all 5 had abdominal pain, and 4 had symptoms of anaemia. Upon physical examination, all patients had pallor, splenomegaly and hepatomegaly. All HMS patients had severe anaemia with a median (IQR) haemoglobin concentration of 6.4g/dl (5.0-7.5) and significantly raised serum IgM, which were well above 2SD above the local mean. Other causes of massive splenomegaly were also determined; 37 had chronic myeloid leukemia, 16 had lymphoma, 13 had chronic lymphocytic leukemia and 8 had acute leukemia. In 6 patients a clear diagnosis was not made and SCD in 4 patients.
Conclusion: These data confirm that, like in other malaria endemic countries, HMS exists in Tanzania. The fact that HMS only accounts for 5% of cases underscores the importance of a systematic approach to the differential diagnosis of massive spenomegaly. This work supports the notion that a very high (non-monoclonal) serum IgM level is the key to diagnosis.