haematology intro Flashcards
What is the difference between a primary and secondary blood disorder
Primary - inherited/acquired disease of the blood
Secondary - normal haem system reacting to non-haem condition
Eg of primary RBC conditions
Raised RBC - polycythaemia vera
Reduced - thalassaemia
Eg of secondary RBC conditions
Raised - high altitude
Low - auto-immune haemolytic anaemia
eg of Inherited primary haematological disorders
Inherited means germline mutation
FIX:
* deficiency > haemophilia B (bleeding tendency)
* Excess > FIX Padua (gene therapy use) thrombosis
Erythrocytes
* Deficiency> B globin gene mutation > Hb S
* Excess > High affinity Hb muation >Erythrocytosis
Acquired primary haematological disorders
Somatic gene mutation - bone marrow has a rapid turnover system
Erythrocytes:
* Excess> JAK2 > Polycythaemia vera
* Deficiency> PIG A > paroxysmal nocturnal haemoglobinuria (PNH)
Eg of secondary factor VIII disorder
Excess > inflammatory response/pregnancy
Deficiency> 2ndry to anti-FVIII auto antibodies (acquired haemophilia A)
Effect of chronic inflammation on blood
raised FVIII levels> increased Thrombosis risk.
Secondary causes of change in Hb
Raised
* {altitude/hypoxia or Epo secreting tumour}
Reduced
* BM infiltration or deficiency {Vit B12, or Fe} disease
* Shortened survival {Haemolytic anaemia}
Secondary causes of changes in plt
Raised
{Bleeding, Inflammation, splenectomy}
Reduced
BM infiltration or deficiency disease {Vit B12 }
Shortened survival {ITP, TTP}
Secondary causes of changes in leukocytes
raised
{Infection, Inflammation, corticosteroids}
Reduced
BM infiltration or deficiency disease {Vit B12 }
Lab findings for Fe deficiency and underlying malignancy
Microcytic hypochromic anaemia
Reduced ferritin, transferrin saturation
Raised TIBC
What is leuco-erythroblastic anaemia
Variable degree of anaemia with
specific morphological features in the blood film
Teardrop RBCs (+aniso and poikilocytosis)
Nucleated RBCs
Immature myeloid cells
What is suggested by a leucoerythroblastic picture
Either - malignant, severe infection, or myelofibrosis
malignant causes of bone marrow infiltration
haemopoietic:
* leukaemia
* lymphoma
* myeloma
non-haemopoietic:
* breast/bronchus/prostate
severe infection causing bone marrow invasion
miliary TB
severe fungal infection
briefly summarise myelofibrosis
massive splenomegaly
dry tap on BM aspirate
primary blood cancer - produce reactive fibrosis around malignant red cells - infiltrates marrow
What are the inherited haemolytic anaemias and the overall cause of each one
Membrane: eg Hereditary Spherocytosis
Cytoplasm/enzymes: eg G6PD deficiency
Haemoglobin: Sickle cell disease (structural Hbpathy) Thalassaemia (quantitative Hbpathy)
Lab results for haemolytic anaemia
Anaemia (though may be compensated)
Reticulocytosis (increased premature RBC) (may cause modest elevation of MCV – because reticulocytes are slightly larger than mature RBC)
Hyperbilirubinaemia (unconjugated/pre-hepatic cause)
LDH raised – intracellular enyme – released from inside the RBC when they are broken down
Haptoglobins reduced
Results for immune haemolytic anaemia - acquired haemolytic anaemiaResults for immune haemolytic anaemia - acquired haemolytic anaemia
Spherocytes - bits of membrane are broken off
DAT +ve (same as coombs test) - detect the Ab that are bound to the red cells.
Associated with systemic diseases:
Imunological disorders
Cancer of immune system: eg Lymphoma or Chronic lymphocytic leukaemia
Auto immune: SLE
Infection: eg mycoplasma
Idiopathic
Types of acquired non-immune haemolytic anaemia
(DAT -ve)
Infection of erythrocytes Malaria
Micro-angiopathic Haemolytic anaemia (MAHA) - associated with systemic disease - RBC broken down in the small blood vessels
Underlying adenocarcinoma
Haemolytic uraemic syndrome
Metal aortic valve
Summarise a microangiopathic film
RBC fragments
thrombocytopenia
Pathophysiology of micro-angiopathy
In adenocarcinomas/low grade DIC
Plt activation
Fibrin deposition and degradation in microvascular
Red cell fragmentation
Bleeding - low plts and coag factor consumption
White blood cells in the bone marrow
Blasts (myeloid & Lymphoid)
Promyelocytes
Myelocytes
If blasts are >5% of bone marrow cells - consider leukaemia or myelodysplasia
What should you consider if bone marrow cells are in the blood
Leukaemia
Met cancer invading bone marrow