2. THE TERM
Cytopenia reduction in either of the cellular component
of blood
• Pancytopenia: Reduction in all the cell lines of blood
The values of the 3 components being:
• Hb <13.5(M)/ 11.5(F) g/dl
• TLC< 4000/cu mm
• Platelets <1,50,000/ cu mm
• Absolute neutrophil count <1500/cu mm
3. It can occur due to
• Bone marrow failure
A. Marrow space occupying lesions
B .Ineffective marrow production
• Peripheral destruction of hematopoietic cells
10. Points to consider in history
• Age- inherited cause of bone marrow failure
• Duration of symptoms-tells about the severity
• Bone pains(acute leukemias) fever(infections), night
sweats(Hodgkins disease), malaise,
weightloss(tuberculosis,malignancy)
• Bleeding from any site(magnitude of thrombocytopenia)
11. • Jaundice(hepatitis viruses)
• Joint pain, rash, photosensitivity(lupus)
• Any radiation exposure
• Exposure to potentially toxic chemicals
• Treatment history including herbals and drug intake, blood
Transfusions
• Dietary history
• Occupational exposure history
12. Clinical Examination
• Anthropometry including short stature in fanconi anemia
• Dysmorphic features (Fanconi anemia)
• Pallor(severity of anemia ), (hepatitisvirus) ,
Lymphadenopathy (leukemia), Edema sign of CHF
• Stomatitis, cheilitis (neutropenia nutrtional deficiency)
• Nail dystrophy, leukoplakia, skin
pigmentation(DYSKERATOSIS CONGENITA)
24. CELULARITY OF BONE
MARROW
The differential diagnosis of pancytopenia are based on
cellularity of bone marrow :
Hypocellular: excessive amount of fat cells
Normocellular: 50-70% hematopoietic cells & 30-50% fat
Hypercellular: 80-100% cells with little fat
25. BONE MARROW
EXAMINATIONAlmost always indicated in cases of pancytopenia unless
cause is apparent
Both aspiration and biopsy are indicated
Specifically, bone marrow aspirate permits examination of:
• Cytology (megaloblastic change, dysplastic changes,
abnormal cell infiltrates)
• Immunophenotyping : antigen or marker on cells
surfaces e.g ( leukemias, lymphoproliferative disorders)
• Cytogenetics : structure of chromosome
(myelodysplasia, leukemias, lymphoproliferative
disorders)
29. ANA test Systemic Lupus Erythematous
BLOOD CULTURE Infectious agent- Tuberculosis or virus
VITAMIN B12 AND FOLATE ASSAYS Megaloblastic anemia
LFT Evaluate hepatitis
KFT Assess for Chronic Renal Failure
SEROLOGY HIV, EBV, Hepatitis
HAM’S TEST Paroxysmal Nocturnal Haemoglobunuria
CHROMOSOMAL BREAKAGE
STUDIES
Fanconi anemia
TEST RATIONALE
BONE X-RAYS Multiple myeloma, metastasis
30. INITIAL MANAGEMENT OF
PANCYTOPENIA
•Discontinue any potential affending drug and use an alternative
class of agent if essential
•Anemia transfusion of leukocyte depleted irradiated red cells as
required for severe anemia.
•Very severe thrombocytopenia or bleeding consider gamma
aminocaproic acid transfusion of platelets as required.
•Severe neutropenia use infections precautions.
31. •Fever(suspected infection, microbial cultures broad
spectrum antibiotics if specific organism not identified.
•If infection is profound and not covered by antibiotic then
consider neutrophil transfusion from a G-CSF.
•Immediate assesment from allogenic stem cell
transplantation. Histocompatibility testing of patient,
parents and siblings. Search databases for unrelated donor,
if appropriate .
33. SUPPORTIVE CARE
Red Cell Transfusion
Packed red cells to alleviate symptoms of anemia usually
are indicated at hemoglobin values below (8g/dl).
•Should be leukocyte-depleted to lessen leukocyte and platelet
sensitization and to reduce subsequent transfusion reactions and
radiated to reduce the potential for a transfusion-related graft-
versus-host reaction.
•It is important to transfuse patients with red cells (or platelets)
from family members if transplantation within the family is
remotely possible.
34. PLATELET TRANSFUSION
Most patients tolerate counts of 10,000/microlitre
(10-109/L)without undue bruising or bleeding, unless a
systematic infection is present or vascular integrity is impaired.
A traumatic injury or surgery requires transfusion to greater than
50,000/microlitre or greater than 100,000/microlitre respectively.
Administration of gamma aminocaproic acid ,50 mg/kg per dose
every 4hours orally or intravenously, may reduce the bleeding
tendency.
Patients should also get ABO-identical platelets because this
enhances platelet survival and further decreases refractoriness to
platelet transfusion
35. MANAGEMENT OF NEUTROPENIA
•Level of neutrophils requiring precautions is fewer than 500/microL
•Neutrophils can be increased by adminstering granulocyte
Colony –stimulating factor
