- M. Amin, a 13-year-old boy, presented with fever for 20 days, vomiting blood for 15 days, and bleeding gums for 15 days. His examination showed pallor and low blood counts. - Investigations revealed pancytopenia, low bone marrow cellularity, and no abnormal cells. This supported a diagnosis of Fanconi's anemia. - Management included controlling infections, correcting anemia with blood transfusions, and immunosuppression with steroids, antilymphocyte globulin, and cyclosporins. Bone marrow transplantation was not indicated due to the patient not meeting criteria of very low blood counts.

2. Presenter
Roll No. 83

3. 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

4. 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

5. 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

6. 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

7. History
• Past history
• Family history
• Social history
• Environmental history
• Personal history
• Drug history

8. • thanks

9. Presenter
Tanzila
Roll No. 16

10. 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

11. Examination
• Weight 32 kg
• Height 142 cm
• Pallor positive
• Other findings negative

12. Examination
• GIT
• CNS
• CVS
• Resp.
– All findings are normal

13. • thanks

14. Presenter
Rana Nasir
Roll No. 176

15. 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)

16. 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

17. 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

18. 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

19. Investigations
• HbS antigen –ve
• Anti HCV -ve

20. • thanks

21. Presenter
Junaid Ahmad
Roll No. 195

22. Management
• Specific Treatment
– Bone marrow transplantation
– Immunosuppression
• General Treatment
– Control of infections
– Correction of anemia
– Correction of bleeding
– Others

23. 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

24. 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

25. 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

26. Management
• Immunosupression therapy
– Steroids
– ALG
– Cyclosporins

27. 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

28. 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

29. Management
• Cyclosporin
– 8mg/kg/day for 14 days
– 15mg/kg/day for three months

30. 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

31. 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

32. Management
– Others
• Hamatopoitic (CSF)
• Recombinant Interlukins (IL-3)
• Q zone (I/V Ig) BD
• Anti thymocytic globulin (ATG)
• Androgens
• Cyclophosphamide reserved for resistatn pts