Haem Flashcards
ALL:
- Most common in children less than 5 years old, associated with Down’s Syndrome
CLL:
Smudge cells, Warm haemolytic anaemia, Richter’s transformation(from CLL to an aggressive high grade b-lymphoma
AML:
Auer rods and high number of blast cells, transformation from a Myeloproliferative disorder(eg. polycythaemia vera)
CML:
Philadelphia Chromosome, has 3 phase: long chronic(several years), accelerated(blast cells take up proportion of blood cells), blast phase
Leukaemia presenting feature:
- Bleeding under the skin due to thrombocytopenia causing non-blanching lesions
Leukaemia Investigations:
- FBC within 48 hours
- Bone marrow Biopsy= for definitive diagnosis (Gold standard)
Leukaemia general mx:
- Chemotherapy
- Targeted therapy(mainly used in CLL- tyrosine kinase inhibitors eg. ibrutinib and monoclonal antibodies eg. rituximab)
Haemophilia: (2 types, inherited X–linked recessive blood disorder therefore mainly affect Males as only have 1 chromosome
A- deficiency in Factor 8
B(Christmas Disease)- deficiency in Factor 9
Haemophilia Presentation:
is a severe bleeding disorder; can result in spontaneous bleeding without trauma and bleeding from eg. internal organs, into joints, intracranial haemorrhage
Haemophilia Inv:
Bleeding score, coagulation factor assays, genetic testing
Haemophilia Mx:
- Give Clotting factors 8/9 depending on type as an IV infusion.
Von Willebrand disease:
- Is the most common inherited cause of abnormal and prolonged bleeding
-deficiency of Von-Willebrand factor(important in platelet adhesion and aggregation) - 3 types: 1(partial deficiency), 2(reduced function), 3(complete deficiency)
Mx: Tranexamic acid for mild bleeding, Desmopressin
Thrombocytopenia= Low platelet count:
either:
- reduced platelet production
- increased platelet destruction
ITP(Immune Thrombocytopenia Purpura):
TTP(Thrombotic Thrombocytopenia Purpura):
ITP: Antibodies are created against platelets, presents with Purpura (non-blanching reasons due to bleeding under the skin). Mx: Prednisolone(steroids), IV immunoglobulin, Rituximab(monoclonal antibody that targets B cells- good for autoimmune related conditions.
TTP:
- Tiny thrombus develop through blood vessels using up platelets. Causes: Thrombocytopenia, purpura, tissue ischaemia/end organ damage
(ADAMTS13 protein),
Myelodysplastic syndromes:
- Form of cancer caused by mutation of myeloid cells and has the potential to transform into AML.
-Presents with: Anaemia, Neutropenia(low neutrophils), Thrombocytopenia-bleeding and purpura (low platelets)
Inv: Bone marrow biopsy is required to confirm diagnosis
Myeloproliferative disorder:
-Uncontrolled proliferation of a single type of blood cell, could transform into AML
- JAK2 mutation
- Main ones to remember(3):
1) Primary Myelofibrosis- low Hb, Mx: JAK2 inhibitor, supportive mx, chemotherapy
2) Polycythaemia Vera- High Hb, gout and thrombosis is a complication of the condition
3) Essential thrombocythaemia High platelet
Blood film: Tear-dropped shaped RBC, Anisocytosis(varying sizes of RBCs), Blasts (immature red and white cells)
Polycythaemia Vera (type of myeloproliferative disorder):
- Red face, conjunctival plethora(red insides of eye), splenomegaly, hypertension
- Bone marrow biopsy to confirm diagnosis
Mx: venesection, aspirin, chemo(hydroxycarbamide)
Myeloma:
Reversal agent for Dabigratran(anticoagulant):
-Idarucizumab
-Other reversal agent: Protamine to reverse=heparin, Vitamin K=warfarin
Sickle cell disease definitive diagnostic inv:
haemoglobin electrophoresis
Transfusion reactions presentations:
Transfusion Associated Circularory Overload(TRACO): HYPERtension, raised jugular venous pulse, AFebrile, S3 present.
Transfusion Related Acute Lung Injury(TRALI): HYPOtension, pyrexia(Febrile), normal/unchanged JVP
Tumour lysis syndrome reduce risk:
Allopurinol or Rasburicase= to lower uric acid in the blood.
Tumour lysis syndrome: Hyperuricaemia, Hyperkalaemia, Hyperphosphate, Hypocalcaemia
- Burkitt’s Lymphoma(fast growing b-cell Non-Hodgkin’s Lymphoma)= common cause of Tumour Lysis syndrome
Post-thrombotic syndrome:
- slow progression of pruritus, pain and swelling are complications that can occur following a DVT.
Why are irradiated blood products used in blood transfusions?
- they are depleted in T cell lymphocytes so it reduces the risk of graft vs. host disease
Target cells, Howell Jowell bodies seen in what condition/presentation:
- coeliac disease= Hyposplenism
Myeloma clinical presentation:
Hypercalcaemia, renal failure, high total protein(could be high proteinuria)
Hodgkin’s lymphoma staging:
1: single lymph node is involved
2: more than 1 lymph node, same side of diaphragm
3: more than 1, both sides of diaphragm
4: spread beyond lymph nodes
Multiple Myeloma presentation: CRAB=
C- HyperCalcaemia
R- Renal failure
A- Anaemia
B- Bone fractures/lytic lesions
Sickle cell crisis: Painful (vaso occlusive: diagnostic Inv=
- Clinical diagnosis and no additional testing is required.
Non-haemolytic febrile transfusion reaction:
Mx: Paracetamol can be used to reduce the pyrexia(fever), also need to stop the transfusion.
Heinz bodies are seen in what condition?
- G6PD deficiency
- Alpha Thalassaemia
Target cells(Bull’s eye spot):
- Iron deficiency anaemia
- Post-Splenectomy
Howell-Jolly bodies:
- Non-functioning spleen
-severe anaemia
Smudge cells (ruptured WBC) :
- Associated with CLL
Spherocytes: (are sphere shaped RBC without bi-concave disk)
- Autoimmune Haemolytic Anaemia
or
-Hereditary Spherocytosis
Anaemia:
- Is defined as low haemoglobin in the blood
- MCV= means size of blood cells and is significant in relation to anaemia
MCV levels in anaemia and type of anaemia correlation:
- Low MCV
- Normal MCV
- High MCV
- Low MCV: Microcytic Anaemia
- Normal MCV: Normocytic Anaemia
- High MCV: Macrocytic Anaemia
Causes of Microcytic Anaemia (TAILS mnemonic):
- T (Thalassaemia)
- A (Anaemia of Chronic disease)
- I (Iron deficiency Anaemia)
- L (Lead Poisoning)
- S (Sideroblastic Anaemia- body produces enough iron but is unable to put it into haemoglobin)
Causes of Normocytic Anaemia (3 A’s and 2 H’s):
- Acute Blood loss
- Anaemia of chronic disease (often occurs with CKD due to reduced production of erythropoietin)
- Aplastic Anaemia
- Haemolytic Anaemia
- Hypothyroidism
Causes of Megaloblastic Anaemia:
- B12 Deficiency
- Folate Deficiency
Generic symptoms and signs of Anaemia:
Symptoms:
- Tiredness
- SOB
- Headaches
- Dizziness
- Palpitations
- Worsening of other conditions eg. angina/heart failure
Signs:
- Pale Skin
- Conjunctival Pallor
- Tachycardia
- Raised Respiratory Rate
Symptoms and signs specific to Iron-deficiency anaemia:
- PICA (dietary cravings for abnormal things eg. metal, dirt, soil)
- Hair Loss
Signs:
- Koilonychia (spoon shaped nails)
- Angular Cheilitis
-Atrophic Glossitis
-Brittle hair and nails
-
what should you do for UNEXPLAINED iron-deficiency anaemia?
- OGD or Colonoscopy= to exclude GI cause of bleeding
- Bone marrow biopsy also= to exclude unknown malignancy(leukaemia/myeloma)
Main Haem conditions:
Iron deficiency (Microcytic Anaemia)
Vitamin B12 deficiency (Macrocytic Anaemia)
Folate deficiency (Macrocytic Anaemia)
Haemophilia (Bleeding Disorder)
Deep vein thrombosis
Pulmonary embolism
Complications of blood transfusion
Myeloma:
(including causes of Myeloma- CRAB mnemonic)
- Type of cancer effecting plasma cells in bone marrow, causes high amounts of M protein (M for Myeloma)
- Presence of Bence Jones Proteins in: Urine protein electrophoresis
- CRAB: Calcium (elevated), Renal failure, Anaemia, Bone lesions and bone pain
- Most common complication= Anaemia
-Other conditions that can be caused=Myeloma bone disease, Renal disease - Bone marrow biopsy= is required to confirm diagnosis
- X-ray findings= Raindrop skull/pepper pot skull: refers to multiple lytic lesions seen on x-ray
- Mx: will never be fully cured; disease is relapsing-remitting.
Treatment: Chemotherapy/High-dose chemotherapy
Lymphoma key symptom:
- Lymphadenopathy
Hodgkin’s Lymphoma:
- May experience lymph node pain after drinking alcohol
+ - B symptoms= Fever, weight loss and night sweats
- Reed-Sternberg cells= key finding in biopsy
- Diagnostic investigation: Lymph Node Biopsy
- Additional non-specific symptoms: Fatigue, itching, cough,SOB, Abdominal pain, Recurrent infections
Management: - Hodgkin’s Lymphoma= Chemotherapy and Radiotherapy
4 stages of Lymphoma (Lugano Classification):
Stage 1: Confined to ONE node/group of nodes
Stage 2: More than group, but on the SAME SIDE of the diaphragm
Stage 3: Lymph nodes ABOVE and BELOW diaphragm.
Stage 4: Widespread involvement, including non-lymphatic organs.
Management for: TACO (Transfusion Associated Circulatory Overload)=
- Should be treated with IV loop Diuretics
- Eg. presentation= Patient had blood transfusion and then started getting SOB/reaction to the medication/blood given.
- Is also indicated by crackles at the bilateral lung bases.