HAEM - Normocytic Anaemia Flashcards
How would blood results look in bone marrow failure?
Hb Reticulocytes WBC Platelets All equally low
If there are no abnormal blasts in pancytopenic marrow - what is the diagnosis?
Aplastic anaemia
Which infecction can cause cessation of marrow erythropoesis
Parovirus
Def haemolysis
The breakdown of RBC before the end of their normal life span
Intrinsic causes of haemolysis (3)
Haemoglobinopathies - Sickle cell/thalassaemia
Membranopathies - spherocytosis, eliptocytosis
Enzymeopathies
Extrinsic causes haemolysis (5)
Autoimmune - warm/cold Alloimmune - Rhesus, transfusion/transplant Dx - pencillins Parasites - plasmodium Microangiopathic - DICq
Ix normocytic haemolysis
FBC Blood film Coomb's Hb electrophoresis Enzyme assays Plasma haptoglobin and urinary haemosiderin
Signs of haemolysis on blood count
Anaemia w/ incr MCV
Raised bilirubin
Raised se LDH
Reticulocyte count >2%
What are you looking for: haemolysis and Coomb’s test
RBC coated in ab/complement –> immune cause
Use of Hb electrophoresis in haemolysis
ID different haemoglobinopathies
Use of plasma haptoglobin + urinary haemosiderin in haemolysis
If plasma haptoglobin = reduced
And
Urinary Haemosiderin = present
== intravascular haemolysis
Genetic linkage Sickle cell anaemia
Autosomal recessive
What type of Hb is produced in Sickle cell
HbS
Who gets Sickle cell
people of African origin
What are the 2 Sickle cell genotypes
HBSS
HBAS
Which genotype –> Sickle Cell phenotype
HBSS
What positive is there to having the Sickle cell trait?
Protection from Malaria
Diagnosis Sickle Cell anaemia (3)
Guthrie
Sickle cells blood film
Hb electrophoresis (confirms)
Sx SIckle cell anaemia - first few m of life
Anaemia
Acute haemolytic crisis –> bone infarcts + painful dactylitis
What can happen if Acute haemolytic crisis is not treated in SickleCell disease
Splenic infarction –> hyposplenism
Repeated renal infarction –> CKD + CVA
Complications of having Sickle cell disease (6)
Hyposplenism CKD Bone necrosis Chronic leg ulcers Fe overload LT pulmonary damage
Chronic treatment for Sickle Cell disease
Lifelong folate
Pneumococcal vaccine + prophylactic penicillin
Hydroxcarbamide
Life long transfusions + Fe chelate
Cure Sickle cell disease
BM transplant
Why do vasoocclusive crises occur in Sickle cell?
B/C microvascular occlusion
PS vasoocclusive crises occur in Sickle cell (4)
SEVERE PAIN Sometimes : Mesenteric ischaemia Cerebral infarctions Priapism
Precipitants: vasoocclusive crises occur in Sickle cell (4)
Cold
Infection
Dehydration
Hypoxia
Cause aplastic crisis
Parovirus B19
mx aplastic crisis
Usually self limiting
+/- transfusion
Who gets sequestrian crises
Mainly children
Sequestrian crises
Pooling of blood in spleeen/liver –> organomealy, anaemia + shock
Mx sequestrian crises
Urgent transfusion
2 examples of enzymopathies
G6PD deficiency
PK deficiency
genetic linkage G6PD deficiency
X linked
Which population suffers from G6PD deficiency?
African/med
Precipitants - G6PD attacks (5)
Aspirin Primaquine Sulphonamides Broad beans Illness
Diagnosis G6PD deficiency
Enzyme assay 3 m after crises
Tx G6PD defic
Ppt avoidance
Transfusion if severe
Cells on film G6PD deficiency
Bite cells + blister cells
Genetic linkage PK deficiency
Autosomal recessive
PS PK deficiency
Neonatal jaundice
–> chronic jaundice + HS megaly
Diagnosis PK deficiency
Enzyme assay
Mx PK deficiency
Well tolerated
Hence not needed
Genetic linkage Hereditary spherocytosis
Autosomal dominant
RBC appearance Hereditary spherocytosis
Spherical
Why do you get Sx in Hereditary spherocytosis
Spherical RBC = < deformable
Get trapped in spleen –> haemolysis, jaundice and splenomegaly
Genetic linkage Hereditary Eliptocytosis
Autosomal dominant
RBC appearance Hereditary Eliptocytosis
Ellipse/oval shaped
Sx Hereditary Eliptocytosis
Mostly asymp
Mx for membranopathies
Folate
Splenectomy - curative but only in severe cases
e.g.s of Coomb’s +ve extrinsic causes of haemolysis (2)
Autoimmune haemolytic anaemia AHA
Drug induced haemolysis
Cause AHA
Commonly idiopathic
Or 2’
to lymphoproliferative disorders
How are AHA’s classified
On optimal Temp antibodies bind to RBC in vitro
What are the 2 types of AHA
Warm AHA
Cold AHA
Binding temperature warm AHA
37’
Which immune Ig is warm AHA mediated by?
IgG
Mx warm AHA
steroids
+/- immunosuppressants
+/- splenectomy
Binding temperature cold AHA
4’
What condition is cold AHA associated with?
Raynauds
Which immune Ig is cold AHA mediated by?
IgM
Mx cold AHA
Cold avoidance
Chlorambucil
Drug induced haemolysis - pencillins
Formation RBC antibodies
Drug induced haemolysis - quinine
Production of immune complexes
Infection –> Coomb’s -ve haemolysis
Malaria/Parasites
E.g.s of microangiopathic anaemia (6)
Malig HTN/pre-eclampsia HUS TTP DIC SLE/vasculitis Mechanical heart valve
Blood film appearance microangiopathic anaemia
Schistocytes
Which category do alloimmune reactions haemolysis fall into?
Immune mediated
But
Coombs negative
What anaemia levels before surgery req transfusion?
<60g/L