Presenter

Roll No. 83

History

• M. Amin 13 years old son of Bashir

Ahmad male Muslim unmarried R/O

Yazman admitted to paeds 2 on 30th

June 2007 with following presenting

complaints

• Fever 20 days

• Hametemesis 15 days

• Bleeding from gums 15 days

History

• HOPI

– My pt was alright 20 days back when he

developed fever. It was gradual in onset

severe intermittent increased on nights

relieved by medication. Fever was

associated with vomiting, gen body aches

and pains, breathlessness on exertions

and headache. Vomiting was scanty

History

– yellow in colour with food contents and

blood drops black in color two to three

times a day, no foul smell. There is also

history of bleeding from gums, two to three

drops two to three times a day then they

went to a local doctor and investigation and

blood test was done.

– They took medicine and blood

History

– transfusion was also done 3-4 pints up till

now. There is also history of wt loss, yellow

color urine and black stools,

– There is no history of jaundice and

lymphadenopathy, abdominal pain,

distension, dizziness, etc

History

• Past history

• Family history

• Social history

• Environmental history

• Personal history

• Drug history

• thanks

Presenter

Tanzila

Roll No. 16

Examination

• GPE

– Pale looking boy with average build and

height having brenula on Rt. hand lying on

bed comfortably well oriented in time

person and place with vitals

– Pulse 110 Bp 125/70

– R/R 20 Temp 98.8

Examination

• Weight 32 kg

• Height 142 cm

• Pallor positive

• Other findings negative

Examination

• GIT

• CNS

• CVS

• Resp.

– All findings are normal

• thanks

Presenter

Rana Nasir

Roll No. 176

Investigations

• Blood examination

– Hb 6.8g/dl (14-18g/dl)

– ESR 35 in first hr (0-12)

– TLC 4300 (4000-11000)

– DLC

• Neutrophils 75% Lymphocutes 22%

• Eusinophils 1% Monoytes 2%

• Basophils 0%

– RBCs 2.5 million (4.5-6.0)

Investigations

• Reticulocytes 0.5%

• Platelets 23000

– PCV 22% MCV 89fl

– MCH 27pg MCHC 30

• Coagulation profile

– Bleeding time increased

– CT normal

– PT normal

– APTT normal

Investigations

• Peripheral blood smear

– Shows normocytic normochromic

• Bone marrow biopsy

– Hypocellularity (all series)

– No abnormal cells

– Marrow space is replaces with fat

• Blood culture

– To rule out any infection

Investigations

• Ultra sound

– Of abdomen to see hepato spleenomegally

– Lymphandenopathy

– Fanconi’s anemia

• LFTs

– Billurubin 0.9mg/dl

– ALT 105 IU/L

– Alkaline phosphatase 254 IU

Investigations

• HbS antigen –ve

• Anti HCV -ve

• thanks

Presenter

Junaid Ahmad

Roll No. 195

Management

• Specific Treatment

– Bone marrow transplantation

– Immunosuppression

• General Treatment

– Control of infections

– Correction of anemia

– Correction of bleeding

– Others

Management

• Bone marrow transplantation

– Indications (severe anemia)• Corrected Reticulocyte < 0.5%

• Neutrophils < 500/uL

• Platlets < 20,000/uL

• Bone marrow cellularity < 20%

– Three out of four required

• Expensive

• HLA compatibility required

Management

• Calculations for corrected Reticulocyte

count• (Observed PCV/Normal PCV) X Reticulocyte

count

• (22/45) X 0.5% = 0.24

• Calculate Neutrophils• TLC X Neutrophil %age

• 4300 X 75% = 3225

Management

• Bone marrow transplantation

– Indications (severe anemia)• Corrected Reticulocyte = 0.24% (<0.5%)

• Neutrophils = 3225/uL (> 500/uL)

• Platlets = 23,000/uL (> 20,000/uL)

• Bone marrow cellularity > 20%

Indications not met

• Expensive

• HLA compatibility required

Management

• Immunosupression therapy

– Steroids

– ALG

– Cyclosporins

Management

• Steroids

– Methyl prednisolone

• 5mg/kg/day for 8 days

• 1mg/kg/day for 6 days

• Taper the dose in next 6 days

• Discontinue the therapy

Management

• ALG and steroids

– Anti leucocytic globulin

• 40mg/kg/dose I/V infusion over 12hrs

– Methyl prednisolone

• 1mg/kg/day I/V for 4 days

Management

• Cyclosporin

– 8mg/kg/day for 14 days

– 15mg/kg/day for three months

Management

• General management

– Control of infections

• Isolation (from inside to outside)

• Antibiotics I/V

– Gentamycin 50mg I/V BD

– According to infections

– No local abscess in neutropenia (Blood cultures)

– Cephlosporins are essential if TLC < 1500

• Anti fungal and Anti virals as needed

Management

– Correction of anemia

• Transfuse blood to raise Hb

• Transfuse packed RBCs

• Take care of nutrition

– Correction of bleeding

• Transfuse platelets (difficult)

• Vit. K 20IU on alternate days

Management

– Others

• Hamatopoitic (CSF)

• Recombinant Interlukins (IL-3)

• Q zone (I/V Ig) BD

• Anti thymocytic globulin (ATG)

• Androgens

• Cyclophosphamide reserved for resistatn pts