| Case Presentation | No. 01-2018 | A 7 year old girl with anemia and massive hepatosplenomegaly Mohammad Mizanur Rahman, Md. Mehedhi Hasan Shourov, Debashish Saha, Md. Abdul Ali Miah and S. M. Motahar Hossain Article Info Presentation of Case advised to get her admitted into the children ward for further management. In the child Departet of Heatology, Ared Dr. Md. Mehedhi Hasan Shourov: A 7 year old ward, patient and her parents were thoroughly Fores Istitute of Pathology, Dhaka Catoet, Dhaka, Bagladesh MMR, girl reported to the child outpatient department interviewed and the child was re-examined. MMHS; Departet of Bioheistry, of a military hospital in Chittagong (South-East The indoor physician ordered initial routine Dhaka Catoet, Dhaka, Bagladesh part of Bangladesh) Cantonment with the com- investigations such as complete blood count, DS; Departet of Mediie, Co- plaints of generalized weakness, loss of appe- peripheral blood film examination, malaria ied Military Hospital, Dhaka, Bagla- desh MAAM, SMMH tite, gradual distention of the abdomen and parasite, immuno-chromatographic test for weight loss. malaria, random blood sugar, liver function tests, urine routine and microscopic examina- The child was reasonably well and performing tion. After getting the results of all investi- For Correspondene: all her daily activities at her own 1 year before. Mohaad Mizaur Raha gations, child specialists sat together, reviewed She was also going to the school regularly and [email protected] her history, physical findings and the results of was worried when her parents noticed the all investigations so far received, discussed the Reeied: Otoer distension of her abdomen and reluctant to take case in details and decided to refer the patient Aepted: Jauary food adequately. The child also developed Aailale Olie: Marh to a tertiary care military hospital at Dhaka for weakness and witnessed weight loss. At that further evaluation, proper diagnosis and time, there was no history of fever, jaundice, management. ISSN: - Olie vomiting, hematemesis and melena. She never - Prit got admitted into the hospital in the past. She In mid June 2015, the patient got admitted into DOI: ./suj.i. was immunized as per national immunization the child ward of a tertiary care military schedule and her milestones of development hospital, Dhaka. The attending child specialist were age proportional. The patient is the first re-examined the patient and reviewed all the Keyords: Aeia; Lier; Splee child of non-consanguineous parents, with investigations performed in Chittagong from normal per vaginal delivery and her only other where the case was referred. The pediatrician Cite this artile: sister who is one year of age is apparently also advised to repeat all the investigations so Raha MM, Shouro MMH, Saha D, normal. There were no signs and symptoms of far done and added some other investigations Miah MAA, Hossai SMM. A year old mental retardation and her neurological func- girl ith aeia ad assie hepato- such as immunochromatographic test for Kala- spleoegaly. Bagaadhu Sheikh tions were intact. She did not receive any blood azar, Mantoux test, serum lipid profile, serum Muji Med Ui J. ; : -. transfusion. As her father has been serving in iron profile, hemoglobin electrophoresis, X-ray Chittagong as a vehicle driver, so the whole chest (P/A view), skull (A/P and lateral view), family was residing in Chittagong though their knee joint (A/P and lateral view), ultrasono- Copyright: The opyright of this artile is retaied permanent residence is in Lalmonirhat (North- graphy and computed tomography of whole y the authors [Atriutio CC-By .] West part of Bangladesh). abdomen and bone marrow examination. On the basis of history, physical findings and On examination in the child outpatient Depart- investigations done at Chittagong, a provisional Aailale at: ment, the patient was found moderately anemic diagnosis was made. .aglajol.ifo and had massive hepatosplenomegaly. There A Joural of Bagaadhu Sheikh Muji were no stigmata of chronic liver disease. Medial Uiersity, Dhaka, Bagladesh Examination of the alimentary system revealed non-tender distended abdomen. Liver was Provisional Diagnosis palpable about eight centimeters from the right Hereditary hemolytic anemia costal margin in mid clavicular line. Spleen was also palpable about 18 cm from the left costal margin in mid clavicular line. Both liver and Differential Diagnosis spleen were non-tender, firm in consistency and have smooth surface with regular margin. Dr Mohammad Mizanur Rahman: This 7 year old There were no evidences of ascities. No abnor- child had a history of anorexia, weight loss, mality was detected on examination of the generalized weakness, gradual distension of the cardiovascular, respiratory, nervous and mus- abdomen, moderate anemia and long standing culoskeletal systems. The attending physician painless massive hepatosplenomegaly. These 50 BSMMU J 2018; 11: 49-54 findings are relatively common in such a child in Table I Bangladesh and there are several differential diag- noses. However, findings such feature in a child of Laboratory data this age particularly in Bangladesh and Indian Subcontinent can help to narrow the list of the Variable Reference range, In the children ward, possible causes. Therefore, I will first focus on my Children (6–12 years)¶ Dhaka differential diagnosis. Hemoglobin (g/dL) 11.5–15.5 7.4 Hematocrit (L/L) 0.4–0.5 0.2 Tropical splenomegaly syndrome RBC (x1012/L) 4.0–5.2 2.5 Tropical splenomegaly syndrome, also known as Mean cell volume (fl) 77–95 69 hyper-reactive malarial syndrome, occurs due to White cell count (x109/L) 5.0–13.0 2.2 frequent assault of malaria infection over a long 1 Differential count (%) period leading to immunological over stimulation. This condition is most prevalent in many malarious Neutrophils 53 44 zones of the tropics including Uganda, Nigeria, Lymphocytes 39 47 New guinea and Congo as well as Indian subconti- Monocytes 04 04 nent.2 Tropical splenomegaly syndrome is described Eosinophils 02 05 by massive splenomegaly, hepatomegaly, marked Basophils <1-2 00 elevations in serum IgM levels (more than 1000 IU/ Blasts 00 00 mL), antimalarial antibodies and a good clinical response to antimalarial therapy with regression of Platelet count (x109/L) 170–450 65 splenomegaly, elevation of hemoglobin level and Peripheral blood film Pancytopenia decreasing serum IgM level.3 Tropical spleno- ICT for malaria Negative megaly syndrome is not the result of active malarial ICT for Kala-azar Negative infection, though it appears that malaria is primari- Liver function tests Normal ly responsible for tropical splenomegaly syndrome. A peripheral smear for malaria parasite is usually Random plasma sugar 5.3 mmol/L negative and the malarial pigment is not found in Urine R/M/E Normal the biopsy material from the liver and spleen. The Mantoux test Negative continual presence of malarial antigen leads to an Serum lipid profile High serum triglycer- aberrant host response resulting in a reactive and ide (193 mg/dL); relatively benign lymphoproliferative disorder that low serum cholesterol affects the liver and spleen, seems more likely to be Serum iron profile Normal the pathogenesis of tropical splenomegaly syn- 4 Hb electrophoresis Normal drome. The condition may show the characters of severe hypersplenism including anemia and throm- Bone marrow study Suggestive of Gaucher bocytopenia.5 As the patient presented with long disease standing painless massive hepatosplenomegaly and ¶ Reference values are affected by many variables, including the demographics and the moderate anemia, these features are, therefore, laboratory methods used. The ranges used at the Armed Forces Institute of Pathology suggestive of tropical splenomegaly syndrome but (AFIP) are for children (6–12 years) who do not have medical conditions that could affect the results. They may, therefore, not be appropriate for all patients no past history of malaria as well as not residing in the malarial hyperendemic zone disfavors the diagnosis of tropical splenomegaly syndrome. Chronic visceral Leishmaniasis Leishmaniasis is a parasitic disease caused by Leish- mania donovani complex (comprising L. donovani, L. infantum, L. chagasi) that spread to people through the bite of the female phlebotomine sandfly.6 Leishmania infection in human is caused by more than 20 species of Leishmania. The risk factors which may predispose to such infections include: Poverty, malnutrition, deforestation and urbanization.7 In about 98 countries, 4 to 12 million people are infected and two million new cases are infected each year. Between 20 and 50 thousand deaths occur in every year. About 200 million people are residing in areas such as Asia, Africa, south and central America and southern Europe where Figure 1: Leishman stained bone marrow slide showing Gaucher cell (indicated by leishmaniasis is common.8 More than 90% of the arrow) World's cases of visceral leishmaniasis are in India, BSMMU J 2018; 11: 49-54 51 differentiation and the t (9:22) (q34:q:11) leading to Table II formation of BCR-ABL fusion gene. Chronic mye- loid leukemia is uncommon in childhood, accoun- Report of enzyme assay ting for only 2 to 5% of all leukemias. It has incidence of <1 case per 1,00,000 population youn- Beta–glucocerebrosidase value (nmol/hour/mg) Remarks ger than 20 years of age per year.16 Chronic myeloid >4 Normal activity leukemia accounts less than 10% of all leukemias 2–4 Possibility of carrier under the age of 20 years and 7–20% of all