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.

PMH

Vaccination was complete.

BW=3500 gr

Ph.Ex

َََ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.

No LAP.

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.

HIV=Neg.

BMA(second)=non specific myelodysplastic changes without hypocellularity

Urine reducing substance=Neg.

Urine & Blood aminoacid chromatography=NL

CXR=NL  

Abdominal sonography=hepatosplenomegaly

Liver biopsy=non specific hepatitis

Ophthalmic consultation=NL

Bone survey=NL

        What is the patient's possible diagnosis?