Fever & hepatosplenomagally

پروفسور محمد حسین سلطان زاده

      استاد دانشگاه علوم پزشکی شهید بهشتی
     متخصص کودکان ونوزادان
        طی دوره بالینی عفونی از میوکلینیک آمریکا
دبیر برگزاری کنفرانس های ماهیانه گروه اطفال
 دانشگاه علوم پزشکی شهید بهشتی


معرفی : دکترعلیرضا فهیم زاد

فوق تخصص عفونی اطفال
به اتفاق اعضای هیئت علمی گروه کودکان
 بیمارستان مفید



Causes of fever & hepatosplenomegaly

Systemic bartonellosis

Acute disseminated histoplasmosis, coccidiomycosis & blastomycosis



Kala-azar, typhoid fever, salmonellosis

Malaria or babesiosis

Hemophagocytic syndrom(HLH)

Malignant infiltrations

Storage disease

Collagen vascular diseases

X-linked lymphoproliferative disaese(XLP)

Or Duncan disease is X-linked recessive trait characterized by an inadequate immune response to infection with EBV.

The defective gene is on Xq25 known as SH2D1A.

Although antibody deficiency is frequently present, this is really a T & NK cell defect.

Affected male are usually healthy until they acquire EBV infection.

The mean age of presentation is less than 5 yr.

There are 3 major clinical phenotypes: 

   1)fulminant, often fatal infectious mononucleosis(50%).


   3)Acquired hypogammaglobulinemia(25%).

XLP overall has an unfavorable prognosis & 70% of affected boys die by age 10 .

Half of XLP given BMT are currently surviving without signs of the disease.