DR Medhat MRCP

Hemolytic crises :
Rare in sicklers
• Routine investigations during sickle cell crises :
CBC : to establish any acute worsening of anemia ,also may ↑ WBCs in infection
Cross matching: the patient may need transfusion
Cultures: to R/O infection.
Reticulocytic count: ↑ in sequestration & haemolytic crisis but ↓ in aplastic
• Screening for chance of a child of a pregnant mother to have sickle cell disease if
there is strong family history
- Pregnant mother will first be screened for sickle cell carrier status.
- If that test is positive, her partner will be screened, and only if both are positive will
she be offered chorionic villus sampling (8-10 weeks) or amniocentesis (14-16 weeks)
II. Aplastic anemia
- BM damage leading to marked hypocellularity of BM & peripheral pancytopenia.
• Causes (= causes of BM depression):
1) 70-80 % idiopathic.
2) Congenital :
Fanconi`s anemia ( autosomal recessive).
Dyskeratosis congenita (skin pigmentation, nail dystrophy,leucoplakia,BM --)
سؤال old. - Median age of presentation is 8 years
3) Drugs :
Antibiotics : chloramphenicol ,sulphonamides,chloroquine, β-lactam Abx.
Anti-neoplastic : cyclophosphamide,methotrexate,azathioprine,MMF.
Anti-inflammatory : declofenac,endomethacin,piroxicam,naproxen.
4) Toxic : radiation,chemotherapy.
5) Infections :
Viral : hepatitis CMV,HIV,Parvovirus.
Bacterial : mycobacteria.
6) Pregnancy.
• Clinical picture : C/P of the aetiology +
Anemia ( ↓ RBCs).
Recurrent infections (leucopenia).
Bleeding tendency (thrombocytopenia).
Hepatosplenomegaly
(compensatory extramedullary hemopoiesis).
25