22 آبان 1386

CPC با مديريت گروه عفونی، گروه هماتولوژی، گروه گوارش در سالن كنفرانس مركز طبي كودكان تاريخ 21/8/86 برگزار گرديد.CPCبحث کنندگان: گروه عفونی، گروه هماتولوژی، گروه گوارشبرگزار کننده: گروه پاتولوژی شرح حال بیمار در نامه ای به ریاست بخش هماتوپاتولوژی در National Cancer InstituteDear Professor Elaine JaffeHereby I want to represent one of our interesting and bewildering cases in a referral children hospital related to Tehran University of Medical Sciences. A 10 year old boy referred to our hospital due to fever and abdominal distention. He gradually developed abdominal distention and fever since a year ago and was admitted in other centers and received a course of therapy with Glucantime blindly with diagnosis of Kala azar with no recovery of symptoms. Clinical examination of admission revealed icteric sclerae, huge hepatosplenomegaly ( spleen under umbilicus, liver span: 15 cm), no lymphadenopathy detected. Facies was rather marfanoid but endocrinologic consultation was not in favor of this diagnosis.Abdominal sonography revealed large liver and spleen ( 150mm and 230 mm respectively ) with no other significant findings.Laboratory data revealed pancytopenia: WBC: 1000 (differential count no possible, Hg: 8.1, MCV: 76, RBC count: 3.04 X 1000000, HCT: 23, MCH: 26.6, MCHC: 35.6, PLT: 21000. ESR: 15, CRP: +++. No positive cultures detected.Karyotype was : 46XY.AST: 83 ( up to 46 ), ALT: 85 ( up to 49 ), Alkaline phosphatase: 875 ( 180-1200 ), Bilirubin: 6.6 ( direct 2.2 ), other tests were unremarkable. Bone marrow aspiration and biopsy was performed ( S. 1567. 85, the paraffin block and our report is included ).He received packed cell and platelet infusions several times but general condition deteriorated and at last as a last resort splenectomy was performed in order to improve the conditions of patient and help in diagnosis. Splenectomy specimen and liver wedge biopsy is numbered S. 1608. 85 and our report and paraffin blocks are included. After surgery the general condition was better and pancytopenia was improved. The last CBC is : WBC: 3.4X1000, Hg: 9.2, PLT: 188000. Also several tear drop and ovalocytes were seen in peripheral smear. Finally he was discharged after a month with rather good general condition and was instructed to admit regularly to our hospital.After four months he referred with loss of appetite and abdominal distention and was hospitalized. Clinical examination on admission revealed fever T: 38.8 ( axillary, the patient had no complaint of fever in last four months ) and hepatomegaly ( 5 cm subcostal margin, span: 17 cm ).Laboratory data: WBC: 6000 Hg:8.8 PLT: 31000 Normal ALT and AST and normal PT and PTT.Platelet was infused and liver biopsy performed. I send the paraffin block of last liver biopsy ( S.161.86 ) for you but I have not reported it as yet. All of the findings is confusing to me, the clinicians are not satisfied with our report and I beg you to help the patient in order to receive a clear diagnosis and hence appropriate therapy.




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