Aplastic anemia
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Transcript of Aplastic anemia
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
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
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
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
• 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
• 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