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


CC : Fever & hepatosplenomagally

PI: The patient is a 4 months old boy that his problem began from a month before admission(17.6.85) with mild intermittent fever . High grade fever occurred from 10 days before admission.

Due to no response to outpatient antibiotic & continuing of fever & hepatosplenomegally he admitted at pediatric infectious ward of Mofid children hospital.

At beginning of admission the patient´s general condition was not bad & had not poor feeding & reflexes were normal.

IV ceftazidime began & due to positive blood culture of staph. Epidermidis, vancomycine added for about 2 weeks.

Because of severe neutropenia, 5 day G-CSF prescribed, but neutrophils did not rise.

During admission, his hepatosplenomegaly progressed & arrived below umbilicus.

At 14.7.85 the patient got respiratory distress & bleeding & transferred to ICU, but was not intubated & due to anemia & thrombocytopenia & severe disturbance of coagulation( disrupted PT & PTT ) multiple doses of P.C , FFP , PLT & Vit K prescribed & IV ceftazidime & vancomycine began.

After 5 days, the patient with good general condition transferred to pediatric infectious ward & antibiotics continued.

Thereafter his fever stopped but pancytopenia & hepatosplenomegally remained.


Vaccination was complete.

BW=3500 gr


َََAT=39 oc  BP=85/p  RR=44/min  PR=130/min  W=5500 gr

At beginning of admission, auscultation of heart & lung was normal.

Liver palpated 4 cm below costal margine & it´s span was large.

Spleen palpated at the level of umbilicus.

There was BCG scar on his arm.


Growth & development were normal.

Laboratory & diagnostic tests

First   PT=20      PTT=45

First CBC(85.6.17)

The last CBC(85.7.18)

BS=101    Ca=9   Na=130   K=4.5

ALP=843(150-420)    LDH=698(150-360)

Trig.=291(30-86)    Chol=79(<170)

Retic=1.7%   ESR=26   CRP=1+

U/A=NL    U/C=Neg.    CSF=NL

CSF culture=Neg.  B/C=staph epidermidis

Wright=Neg.  2-ME=Neg.   ABG=NL

BUN=6   Cr=0.5   SGOT=54    SGPT=32

Bil=1.2 – 0.3

Malaria & Borelia PBS=Neg.

BMA(first)=NL    IFA(leishmaniasis)=Neg.

IgM CMV=Neg.   IgM & IgG VZV=Neg.


BMA(second)=non specific myelodysplastic changes without hypocellularity

Urine reducing substance=Neg.

Urine & Blood aminoacid chromatography=NL


Abdominal sonography=hepatosplenomegaly

Liver biopsy=non specific hepatitis

Ophthalmic consultation=NL

Bone survey=NL

        What is the patient's possible diagnosis?