mini presentation about pancytopenia and approach to it pancytopenia reduction in number 2 or more of cbc wbc plt itself not a diagnosis it is sign

1. AbbasWael Abbas
Supervisor: Dr. Sultan Musleh

2.  Pancytopenia is a
 reduction in the number of 2 or more
▪ RBCs,
▪ WBCs and,
▪ platelets
 in the peripheral blood
 below the lower limits of the age-adjusted normal
range for healthy people.

3.  Therefore it is the combination of anemia,
leukopenia, and thrombocytopenia :-

4.  It may result from
 decreased production of blood cells or bone
marrow failure,
 immune-mediated destruction
 non-immune-mediated sequestration in the
periphery,
 Deficiency of factors stimulating haematopoiesis

5. • The diagnosis is made from the results of CBC,

7.  Congenital
 Fanconi’s Anemia
 Shwachman-Diamond syndrome

8.  Congenital aplastic anemia
 Etiology. autosomal recessive, defect in proteins
involved in DNA repair
 Clinical features
▪ bone marrow failure occurs at a mean age of 7 years.
Typical presentation is with ecchymosis and petechiae.
▪ Skeletal abnormalities; short stature in almost all
patients, and absence or hypoplasia of the thumb and
radius, microcephaly

9. ▪ Skin hyperpigmentation, café au lait spots
▪ Renal abnormalities; horseshoe or absent
kidney
▪ 10% of cases transform to luekemia

10.  pancytopenia, RBC macrocytosis, low
reticulocyte count, elevated Hgb F, and bone
marrow hypocellularity
 The diagnosis is based on demonstration of
increased chromosomal breakage after
exposure to agents that damage DNA

11.  transfusions of RBCs and platelets as needed
 bone marrow transplant from an HLA-
compatible donor
 Corticosteroids
 Androgenic therapy; 20% of cases

12.  Autosomal recessive
 clinical feature:
malabsorption, short stature caused by metaphyseal
chondrodysplasia, marrow failure and neutropenia.
recurrent infections

13. ▫ Mechanism : Decreased Marrow Production
• Conditions
▫ Cytotoxic Chemotherapy
▫ Radiation Therapy
▫ Megaloblastic anemia
▫ Bone Marrow Infiltration
▫ Viral Infections like CMV, EBV, HIV
▫ Hypersplenism
▫ Idiopathic Aplastic Anemia
▫ SLE, RA

14.  ‫والثالث‬ ‫التستماع‬ ‫حسن‬ ‫والثاني‬ ‫الصمت‬ ‫العلم‬ ‫أول‬
‫نشره‬ ‫والخامس‬ ‫به‬ ‫العمل‬ ‫والرابع‬ ‫حفظه‬

‫المنفلوطي‬ ‫لطفي‬ ‫مصطفى‬

15.  History
 Clinical Examination
 CBC
 Peripheral smear examination
 Bone Marrow Aspiration
 Bone Marrow Biopsy
 Other specific investigations

16.  Duration of symptoms
 H/o Transfusions
 H/o Haemoglobinuria
 Dietary history
 Family history
 Exposure to –
 Drugs ( sulfonamide, anticonvulsant, chloramphenicole)
 Chemicals
 Radiation
 Infections
 Weight loss, fever – malignancy & inflammatory
 Jaundice – Hep B & C
 Joint Pain – SLE
 Blood Loss

17. • A thorough physical exam is required, preferably by a
haematologist.
• Skin; petechiae, and purpura, hyperpigmintation
• Lymphadenopathy
• splenomegaly.

18. The following reference points to specific organ systems and
associated conditions and is helpful to guide the examination.
• Eye examination
▫ Jaundiced sclera (paroxysmal nocturnal hemoglobinuria,
hepatitis, cirrhosis)
▫ Epiphora (dyskeratosis congenita)
• Oral examination
▫ Stomatitis or cheilitis (neutropenia, vitamin B12 deficiency)
▫ Gingival hyperplasia (leukemia)
▫ Oral candidiasis or pharyngeal exudate (neutropenia, herpes
family virus infections)

19. • Cardiovascular examination
▫ Tachycardia, edema, congestive cardiac failure
▫ Exercise intolerance
▫ Systolic flow murmur
▫ Tachypnea (sign of symptomatic anemia)
• Abdominal examination
▫ Right upper quadrant tenderness (hepatitis)
• Musculoskeletal examination
▫ Short stature (Fanconi anemia, other congenital syndromes)
▫ Swelling/synovitis (SLE)
▫ Abnormal thumbs (e.g., Fanconi anemia)

20. • A CBC and examination of peripheral blood film by a
hematologist are essential. A standard battery of
evaluative tests may include:
▫ Serum reticulocyte count
▫ Serum LFTs
▫ Hepatic serology
▫ Serum coagulation profile, bleeding time, fibrinogen, and D-
dimer
▫ Coombs test
▫ Serum B12 and folate
▫ Serum HIV and nucleic acid testing.

21. Red Cell Morphology
 Normocytic normochromic with no anisopoikilocytosis, – Aplastic anemia
 Macro ovalocytes with Howell Jolly Bodies – Megaloblastic anemias
 Macrocytic – Fanconi anemia
WBCs
 Leucopenia (mostly mature ~80%) – Aplastic anemia
 Neutrophils present in increased number with toxic granules, shift to left –
Infections
 hypersegmented neutrophils – Megaloblastic anemia
 Blasts –leukemia
Platelets
 Giant platelets – MDS/ Hypersplenism

22. Aspiration and biopsy
 Hypocellular
 Cellular
 Bone marrow filtration

24.  thank you