Pancytopenia Flashcards
What is pancytopenia
A deficiency of blood cells of all lineages
Name one inherited cause of pancytopenia
Fanconi anaemia
Primary acquired causes of pancytopenia
Idiopathic aplastic anaemia
Myelodysplastic syndromes
Acute leukaemia
Secondary acquired causes of pancytopenia
Drug induced aplasia
Vitamin deficiency (B12/folate)
Infections
Metastatic cancer
Storage disorders
What are the 2 main mechanisms that cause pancytopenia
Reduced production
Increased destruction / sequestration
What causes increased destruction of cells in pancytopenia
Hypersplenism
What causes reduced production of cells in pancytopenia
Bone marrow failure
Why does fanconis anaemia cause pancytopenia
Patients are unable to correct inter-strand cross links (DNA damage)
Why does idiopathic aplastic anaemia cause pancytopenia
Autoreactive T cells target haemopoiesis
Why do myelodysplastic syndromes cause pancytopenia
Increased apoptosis of progenitor and mature cells
What are the 3 main causes of hypersplenism
Splenic congestion
Systemic disease
Haematological disease
Name one cause of splenic congestion
Portal hypertension
Name one systemic disease that can cause hypersplenism
Rheumatoid arthritis
Name one haematological disease that can cause hypersplenism
Splenic lymphoma
General symptoms of anaemia
Fatigue
SOB
Cardiovascular compromise
General symptoms of neutropenia
Increased severity and duration of infections
Opportunistic infections
Infections with what type of bacteria are the most dangerous
Gram negative
Neutropenia
Low white cell count (neutrophils)
Thrombocytopenia
Low platelet count
General symptom of thrombocytopenia
Bleeding
General investigations for pancytopenia (3)
FBC
blood film
Bone marrow examination
Supportive management of pancytopenia
Transfusions
Antibiotic prophylaxis if needed
What causes hypocellular marrow in pancytopenia
Aplastic anaemia
What causes hypercellular marrow in pancytopenia (3)
Myelodysplastic syndromes
B12/folate deficiency
Hypersplenism
What can be used to confirm aplastic anaemia
Bone marrow biopsy
Inheritance seen in fanconi anaemia
Autosomal recessive
What is the most common cause of aplastic anaemia
Radiation exposure