Pancytopenia Flashcards
What does pancytopenia mean?
A DEFICIENCY OF BLOOD CELLS OF ALL LINEAGES
• Usually refers to erythrocytes, platelets and granulocytes = anaemia/neutropenia/thrombocytopenia
What are the two general causes for pancytopenia?
- Reduced production
- increased destruction
Reduced blood cell production i.e. marrow failure can either be inherited or acquired. What is the cause for inherited bone marrow failure?
Defects in DNA repair/ribosomes e.g. fanconis anaemia (v rare) • Short stature • Skin pigment abnormalities – café au lait spots • Radial ray abnormalities – no radius • Hypogenitilia • Endocrinopathies • GI defects • Cardiovascular • Renal
Haematological
o Median age: 7 years
o Unable to correct inter-strand cross-links (DNA damage)
o Macrocytosis followed by thrombocytopenia, then neutropenia
o Bone marrow failure (aplasia) risk: 84% by 20 years
o Leukaemia risk: 52% by 40 years
Reduced blood cell production i.e. marrow failure can either be inherited or acquired. What are the three primary causes for acquired bone marrow failure?
Aplastic anaemia
o Autoimmune attack against haemopoietic stem cell (therefore no haemopoiesis)
Myelodysplastic syndromes (MDS) o Increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)
Acute leukaemia
o White cell count can be variable
o may or may not present with pancytopenia
Reduced blood cell production i.e. marrow failure can either be inherited or acquired. What are the four secondary causes for acquired bone marrow failure?
ν Drug induced [eg chemotherapy, chloramphenicol, alcohol] – causes aplasia
ν B12/folate deficiency (nuclear maturation can affect all lineages)
ν Malignant: non-haemopoietic infiltration, lymphoma
ν Misc.: Viral (eg HIV)/storage diseases
What are the three causes of increased destruction of blood cells causing pancytopenia?
Hypersplenism
autoimmune e.g. SLE
sepsis - severe bacterial sepsis can cause reduction in blood counts
why does hypersplenism cause pancytopenia?
♦ Increased splenic pool – increase in cells pooling in the spleen and less cells therefore in the blood stream
♦ Increased destruction that exceeds bone marrow capacity, usually associated with significantly enlarged spleen
What are three causes of splenomegaly?
Splenic Congestion
θ Portal Hypertension
θ Congestive cardiac failure
Systemic diseases
θ Rheumatoid Arthritis (Felty’s)
Haematological diseases
θ Myelofibrosis (or other chronic myeloproliferative disorders)
What signs and symptoms are found in pancytopenia due to:
- anaemia
- neutropenia
- thrombocytopenia
Anaemia:
♦ Fatigue
♦ Shortness of breath
♦ Cardiovascular compromise
Neutropenia:
• Infections: severity/duration
(Beware Neutropenic fever)
Thombocytopenia:
♦ Bleeding
♦ Purpura
♦ Petechiae
When investigating pancytopenia marrow cellularity is used, what would cause a hypocellularity vs a hypercellularity?
Hypo - aplastic anaemia
Hyper i.e. Bone marrow is making lots of cells but they can’t get into the bloodstream – ineffective haemopoiesis:
• Myelodisplastic syndromes – increased level of apoptosis
• B12/Folate deficiency – maturation failure
• Hypersplenism – pooling
Describe the supportive treatment for pancytopenia?
o Red cell transfusions o Platelet transfusions o Antibiotics treatment and prophylactic use (empirical for neutropenic fever) ♣ antibacterials* ♣ antifungals ♣ Antivirals
Describe specific treatment for bone marrow disorders:
- malignancy
- congenital
- viral
- idiopathic aplastic anaemia
- drug rxn
♦ Malignancy – consider chemotherapy
♦ Congenital – consider bone marrow transplantation
♦ Viral – eg treat HIV
♦ Idiopathic Aplastic Anaemia – Immunosuppression
♦ Drug reaction – STOP
How is hypersplenism treated?
treat cause if possible, consider splenectomy
