Pancytopenia Flashcards
(20 cards)
Pancytopenia broken down (3)
‘Pan- ’ - Generalised/Whole
‘-cyto-’ - Prefix denoting a cell
‘-penia’ – Lack/deficiency (in blood)
Definition Pancytopenia (3)
a deficiency of blood cells of all lineages
not a diagnosis - reflects a diagnosis
not always mean bone marrow failure/ malignancy
Causes of Pancytopenia (2)
reduced production
or
increased destruction
Reduced production - causes (3)
bone marrow failure
acquired=
-prim
-sec
inherited syndromes- arise due to defects in DNA repair/ ribsosomes/ telomeres
Inherited Marrow Failure Syndromes - RARE (6)
e.g. Fanconi’s anaemia
-Short stature
-Skin pigment + radial ray abnorm
-Hypogenitilia
-Endocrinopathies
-GI defects, Cardio, Renal, Haem
Acquired Primary Bone Marrow Failure (4)
an intrinsic marrow problem
Idiopathic aplastic anaemia: autoimmune attack against haemopoietic stem cell(s)
Myelodysplastic syndromes (MDS)
Acute leukaemia
Myelodysplastic syndromes (5)
Clonal haemopoetic stem cell disorder characterised by dysplasia or unique genetic abnormalities
Hypercellular marrow
Increased apoptosis of progenitor + mature cells (ineffective haemopoiesis)
Not all patients will have pancytopenia – isolated anaemia most common
Propensity for evolution into AML
Why can acute leukaemia cause pancytopenia? (3)
Prolif of ABNORMAL cells (blasts) from leukaemic stem cells (LSC)
Failure to differentiate or mature into normal cells
Prevent normal haemopoietic stem/progenitor (HSC) development by ‘hijacking’/altering the haemopoietic niche + marrow microenvironment
Secondary Bone Marrow Failure (4)
-drug induced= chemotherapy, alcohol, azathioprine, methotrexate- causes aplasia
-B12/ folate deficiency=
-infiltrative=non-haemopoietic malignant infiltration, lymphoma
-misc: viral (eg HIV)/storage diseases
Increased destruction (5)
Hypersplenism=
-increased splenic pool
-increased destruction exceeds bone marrow capacity, assoc significantly enlarged spleen
any cause of splenomegaly can result in hypersplenism
splenic size alone may not correlate with hypersplenism
Causes of Hypersplenism (3)
Splenic Congestion=
-Portal Hypertension
Systemic diseases=
-Rheumatoid Arthritis
Haematological diseases=
-Splenic lymphoma
Clinical features of pancytopenia can reflect
lack of circulating blood cells
cause of the pancytopenia
Clinical features of pancytopenia- 1 (4)
Anaemia=
-fatigue
-SOB
-cardio compromise
Clinical features of pancytopenia- 2 (6)
Neutropenia=
infections- sev + duration
=gram negative sepsis
=gram positive bacteria
=cutaneous inf
=invasive fungal inf
Clinical features of pancytopenia- 3 (5)
Thrombocytopenia=
-Bleeding
=Purpura
=Petechiae
=’Wet’ bleeds including visceral bleeds
Marrow Cellularity in Pancytopenia (6)
Variable depending on cause
Hypocellular = aplastic anaemia
Hypercellular=
-Myelodysplastic syndromes
-B12/folate deficiency
-Hypersplenism
Treatment (7)
Supportive
=Red cell transfusions
=Platelet transfusions
=Neutrophil transfusions not routine
Antibiotics prophylaxis/treatment
=antibacterials, antifungals
Treat neutropenic fever promptly based on local unit antibiotic policy without waiting for (microbiology) results
Treat- Prim bone marrow disorder (4)
Malignancy – consider treatment (chemotherapy)
Congenital – consider allogeneic stem cell transplantation
Idiopathic Aplastic Anaemia – Immunosuppression
MDS -stimulating agents in low risk, demethylating agents or chemotherapy in higher risk
Treat- Sec bone marrow disorder (3)
Drug reaction – STOP
Viral – eg treat HIV
Replace B12/folate
Treat- Hypersplenism
Treat cause if possible
Consider splenectomy (not appropriate in all cases)
