Haematology Flashcards
Which are the ACUTE leukaemia types / classifications?
- Acute lymphoblastic leukaemia (ALL)
- Acute myeloid leukaemia (AML)
What is the epidemiology of ALL
?
Children.
What are the symptoms of ALL
?
- Fatigue (anaemia)
- Bleeding, petechia, purpura, ecchymoses (trombocytopenia)
- Recurrent infections (low or abnormal WBC’s)
- LUQ tenderness & early satiety (splenomegaly).
Diagnosis of ALL (acute lymphoblastic leukaemia)
► Most initial
⟶ FBC depression of all 3 cell lines:
* Anaemia (normochromic normocytic)
* WBC (low, normal or elevated)
* Thrombocytopenia
► Most appropriate
⟶ Peripheral blood test: Leukaemic blasts/lymphoblasts on peripheral blood
► Most definitive test
⟶ Bone marrow biopsy:
* Blasts cells.
What is the epidemiology of AML
?
Mostly elderly.
What are the symptoms of AML
?
Acute myeloid leukaemia
⬇︎ RBC
- Anaemia
- Fatigue
⬆︎ WBC
- Fever
- Leukocytosis / leukopenia
- Gingivitis
⬇︎ PLT
- Bleeding
- Hepatosplenomegaly
Diagnosis of AML
?
► Most initial
⟶ FBC (abnormal)
► Most appropriate
⟶Peripheral smear:
Auer rods
► Most definitive test
⟶Bone marrow biopsy:
Myeloblasts
Where are the following absorbed in the body?
- B12
- Folate
B12
-Terminal ileum
Folate
- Jejunum / duodenum
What are the causes of Vitamin B12 defficiency
?
- Vegan diet
- Gastrectomy
- Pernicious anaemia
- Ileal disease
- Chrohn’s disease
- Malabsorption diseases
What are the symptoms of Vitamin B12 defficiency
?
- Macrocytic anaemia
- Pins and needles on the limbs
- Cognitive changes
What is the Rx of Vitamin B12 defficiency
?
Hidroxycobalamin IM.
DDx of peripheral smear
in leukaemias.
ALL
- Lymphoblasts
AML
- Myeloblast
- Auer rods
CLL
- Small mature lymphocytes
- Smudge cells
CML
- Mature myeloblasts
- ⬆︎ Basophils
What are the symptoms of CLL
?
Chronic lymphocytic leukaemia
- Leukocytosis
- Enlarged lymph nodes
Diagnosis of CLL
Peripheral blood smear
-Smudge cells
What is the epidemiology of CML
?
Chronic myelocytic leukaemia
Elderly.
What are the symptoms of CML
?
- Leukocytosis
- Massive splenomegaly
Diagnosis of CML
Peripheral blood smear
- All stages of maturation seen
- Basophils increased
Genetics
- Philadelphia chromossome
DDx between:
- Von Willebrand
- Haemophilia
- Disseminated intravascular coagulation
Von willebrand
- Platelet type bleeding
- Mucosal bleeding
- aPTT prolonged
- Bleeding time prolongued
Haemophlia
- Factor type bleeding
- Bleeding into joints and muscles
- aPTT prolonged
DIC
- Bleeding everywhere
- aPTT prolonged
- PT prolonged
- Bleeding time prolonged
- ↑ D-dimers
-
aPTT (activated partial thromboplastin time): measures the amount of time it takes for a blood clot to form.
Normal:
21 - 35 sec. -
PT (prothrombin time): measures how many seconds it takes for a clot to form in a blood sample
Normal:
11 - 13 sec.
DDx between:
- Vit B12 deficiency
- Folate deficiency
Vit B12 deficiency
- Vegans
- Found in meats
Folate deficiency
- Meat eaters
- Found in vegetables
DDx between:
- Direct coombs test
- Indirect coombs test
Direct coombs test
- Diagnosis of autoimmune haemolytic anaemia
- Detects antibodies on RBC surface
Indirect coombs test
- Detects antibodies in the serum
- Cross-matching of blood
- Antenatal antibody screen
Describe Disseminated intravascular coagulation.
- Widespread activation of the coagulation cascade;
- Results in ⬇︎ clotting factors;
- Blood clots in different organs;
- Bleeding.
What are the causes of DIC
?
- Sepsis
- Major trauma
- Complications of pregnancy
- Cancer
What are the symptoms of DIC
?
- Bleeding
- Purpura
- Petechiae
What are the investigations / LABS of DIC
?
Increased:
- PT
- aPTT
- INR
- D-Dimer
Decreased:
- PLT
- Fibrinogen
What is the Rx of DIC
?
1. Transfusion
- Fresh frozen plasma
- PLT
2. Treat the cause
What are the causes
of folate deficiency?
➜ Dietary
- Malabsorption (coeliac disease, jejunal resection, Chronh’s disease);
- Poor intake
➜ Drugs
- Methorexate
- Trimetoprim
- Anticonvulsants
- Sulfasalazine
What are the investigations
done in folate deficiency?
◉ FBC
- Anaemia with MCV > 96
- Serum folate: low
What is the RX
of folate deficiency?
- Folic acid 5mg per day for four months.
Describe what is G6PD deficiency
?
- Inadequate levels of glutathione reductase
to protect the red cells from oxidant stress
➝ haemolysis. - X-linked recessive disorder.
Describe the triggers for a haemolytic crisis in G6PD deficiency
?
- Broad beans (favism)
- Primaquine
- Nitrofurantoin
- Sulfonamides
Describe the symptoms in G6PD deficiency
?
- Mostly asymptomatic
- Haemolysis
- Jaundice
Describe the investigations in G6PD deficiency
?
Blood film
- Heinz bodies and bite cells
- Negative coombs test
- ↑ LDH
- ↓ Haptoglobin
Describe the Rx for G6PD deficiency
?
- Blood transfusion and dialysis (severe cases)
- Folic acid
- Avoid triggers
Splenectomy (last resort)
What are the types of Haemophilia
?
➔ Haemophilia A
- Factor VIII deficiency
➔ Haemophilia B (Christmas disease)
- Factor IX deficiency
What is the genetic characteristcs of Haemophilia
?
X-linked recessive disorder.
What are the symptoms of Haemophilia
?
Spontaneous bleeding into:
- Joints
- Muscles
What are the labs investigation in Haemophilia
?
- ↑ APTT
Normal:
- PT
- Von Willebrand factor
What is the treatment of Haemophilia
?
Haemophilia A
- Factor VIII infusion
- Desmopressin (↑ factor VIII)
Haemophilia B
- Recombinant factor IX
What SHOULD BE AVOIDED in Haemophilia
?
1. NSAIDS
- Gastrointestinal bleeding
2. IM injections
- Haematoma
Describe what is hereditary spherocytosis?
- Autosomal dominant condition
Defect in RBC structural protein ➝ spherocytes ➝ haemolysis in the spleen ➝ anaemia.
What are the symptoms
of hereditary spherocytosis?
- Haemolytic anaemia
- Jaundice
- Splenomegaly
- Gallstones
What are the investigations
done in hereditary spherocytosis?
Peripheral smear
- Spherocytes
- Howell-Jolly bodies
Others
- Negative directo coombs test
- Positive osmotic fragility
What will happen if an individual with hereditary spherocytosis or with sickle cell anaemia if he is infected with Parvovirus B19
?
Aplastic crisis.
What is the Rx
of hereditary spherocytosis?
- Folate supplements
- Splenectomy
What are the diseases associated with Parvovirus B19?
- Slapped cheek syndrome
- Hereditary spherocytosis
- Sickle cell anaemia
B19 is the cause of slapped cheek.
- In a hereditary and sickle cell, the patients are dependent on erytrhopoiesis due to short lifespan of RBC’s.
- B19 decreases erytrhopoiesis hence aplastic crisis.
What is Hodgkin’s lymphoma?
Malignant tumour of the lymphatic system.
What are the symptoms
of Hodgkin’s lymphoma?
General
- Painless lymphadenopathy
- Splenomegaly
- Weight loss
- Hepatomegaly
- B symptoms
B symptoms
- Weight loss
- Night sweats
- Fever > 38
- Fatigue
What is the epidemiology
of Hodgkin’s lymphoma?
- < 25 years
- > 55 years
What is the investigation
done in Hodgkin’s lymphoma?
Peripheral smear
- Reed - Sternberg cells (multilobulated giant cells)
Descrine NON-Hodgkin’s lymphoma?
Absence of Reed - Sternberg cells.
What are the risk factors
for Hodgkin’s lymphoma?
- Immunosuppression
- Smoking
What is one of the types of NON
-Hodgkin’s lymphoma?
Burkitt lymphoma
- Associated with HIV.
DDx between:
- Children
idiophatic thrombocytopenic purpura
- Adults
idiophatic thrombocytopenic purpura
Children
idiophatic thrombocytopenic purpura
- Acute
- Viral prodrome
- Lasts for few weeks with complete recover
Adults
idiophatic thrombocytopenic purpura
- Chronic
- No viral prodrome
- PLT doesn’t return to normal
What is the treatment of idiophatic thrombocytopenic purpura
?
- Steroids
- Immunoglobulins
- Splenectomy
What is the cause of thrombotic thrombocytopenic purpura
?
Deficiency of ADAMTS13
- It cleaves the von willebrand factor.
What are the symptoms of thrombotic thrombocytopenic purpura
?
It’s a pentad.
Pentad
- Fever
- Thrombocytopenia
- (Microangiopathic) haemolytic anaemia
- Renal failure
- Neurological abnormalities
What is the labs / investigation of thrombotic thrombocytopenic purpura
?
- Blood smear: Fragmented RBC’s
- ↓ ADAMTS13 levels
- ↑ creatinine
- Microscopic haematuria
- ↑ LDH
What is the Rx of thrombotic thrombocytopenic purpura
?
- Plasma exchange
- Steroids
Relapse:
- Rituximab
What are the causes
of iron deficiency anaemia?
- ↓ intake
- Blood losses from GIT
- Menorrhagia
What are the investigation
done in iron deficiency anaemia?
Decreased:
- Ferritin
- MCV
- HgB
- Transferrin saturation
Increased:
- ↑Total iron binding capacity (TIBC)
What are the symptoms
of iron deficiency anaemia?
- Koilonychia
- Tachychardia
- Pallor
- Angular stomatitis
- Restless legs
- Fatigue
What is the Rx
of iron deficiency anaemia?
- IV iron
- Blood transfusion
What is the association
between milk
and iron deficiency anaemia?
Milk decreases iron absorption.
DDx between:
- Iron deficiency anaemia
- Anaemia of chronic disease
- Thalassaemia trait
Iron deficiency anaemia
- Serum iron: ↓
- Ferritin: ↓
- TIBC: ↑
- Transferrin: ↑
- Transferrin saturation: ↓
Anaemia of chronic disease
- Serum iron: ↓
- Ferritin: ↑
- TIBC: ↓
- Transferrin: ↓
- Transferrin saturation: ↓
Thalassaemia trait
- Serum iron: ⟷
- Ferritin: ⟷
- TIBC: ⟷
- Transferrin: ⟷
- Transferrin saturation: ⟷
Describe Multiple Myeoloma.
- Cancer of plasma cells;
- Infiltrate the bone marrow causing it to replicate the cancerigenous cells.
What are the symptoms
of Multiple Myeoloma?
CRAB
- Hyperc
alcaemia
- R
enal failure
- A
naemia
- B
one pain
What are the investigations
done in Multiple Myeoloma?
-
Urine protein electrophoresis
⟶ Bence Jones protein; -
Serum protein electrophoresis
⟶ increased monoclonal immunoglobulin spike; -
X-ray
⟶ punched out lytic lesions; -
Blood film
⟶ Rouleax formation; -
Bone marrow biopsy
⟶ Abundant plasma cells
What are the step by step investigations
done in Multiple Myeoloma?
First line
- FBC (↓ HgB)
- Renal function (↓ creatinine)
- ↑ Calcium
2nd line
- Serum electrophoresis
- Serum free light essay
- Bence-jones urine protein assessment
Diagnostic tests
- Bone marrow biopsy
- Whole body MRI
(1st line imaging)
- Skeletal survey (when MRI unsuitable)
What diseases are associated with non-blaching rash
?
- Henoch Shonlein purpura
- Idiopathic thrombocytopenia
- Haemolytic uremic syndrome
- Acute leukaemia
- Bacterial meningitis
Describe what is Scurby disease.
Disease resulting from lack of vitamin C.
What are the risk factors
for Scurby disease?
- Elderly
- Neglected individuals
- Chronic alchoolism
What are the symptoms
of Scurby disease?
- Spontaneous haematoma
- Petechiae
- Purpura
- Loose teeth
- Bleeding gums
- Hyperthrophic gums
- Lethargy
- Pallor
How is the diagnosis
of Sickle cell disease made?
- Blood smear: ↑ reticulocytes and sickle cells
- HbG electrophosporesis for HbSS
How is the management
of Sickle cell disease done?
- Folic acid
- Blood transfusions
- Oral penicillin prophylaxis
- Avoid triggers
How is the pain management
in Sickle cell disease made?
Mild pain
- NSAIDS
Severe pain
- Opioids (except pethidine
)
Pethidine leads to accumulation of metabolites in sickle cell and causes neurotoxicity.
What is the management of warfarin with high INR and bleeding
?
Massive bleeding
- Stop warfarin
- Administer phrotrombin complex concentrate
- Administer Vitamin K IV
- Fresh frozen plasma (if no PCC)
Minor bleedINR > 5
- Stop warfarin
- Give Vit K IV
No bleedINR > 8
- Stop warfarin
- Give Vit K oralINR > 8
- Stop warfarin
What are the types of thalassaemias
?
- Alpha thalassemia: die in utero
- Beta thalassemia
What are the symptoms
of Beta thalassemia major?
- Frontal bossing
- Haemolytic anaemia
- Hepatosplenomegaly
- Iron overload (due to repeated transfusions)
What are the investigations
done in Beta thalassemia major?
- ↑ serum iron
- ↑ ferritin saturation
- ↑ ferritin
- Microcytic hypochromic anaemia
- ↑ HbA2 on electrosphoresis
What is the treatment
of Beta thalassemia major?
- Life long blood transfusion
- Iron chelation (prevents overload)
Von Willebrand disease
What is the function of Von Willebrand factor
?
- Facilitates PLT adhesion to endothelial cells
- Carrier molecule for factor VIII
Describe Von Willebrand disease
.
Abnormal function or deficiency of Von Willebrand factor.
- PLT can’t form plug
- Factor VIII degradates
What are the symptoms of Von Willebrand disease
?
- Epistaxis
- Mucosal bleeding
- Easy bruising
- Menorrhagia
What are the investigations done in Von Willebrand disease
?
- Prolongued aPTT
- INR & PT: Normal
- PLT: normal
What is the Rx of Von Willebrand disease
?
- Tranexamic acid
- Desmopressin (releases vWF from endothelium)
Describe what is tumour lysis syndrome.
Massive cell death in chemotheraphy.
Describe the presentation
of tumour lysis syndrome.
- Hyperuricemia
- Hyperkalaemia
- Hyperphosphatemia
- Hypocalcemia
What is the Rx
of tumour lysis syndrome?
- IV fluids
- Diuretics
- Rasburicase
- Kidney failure: haemodialysis
- Allopurinol (prevention)
What is the investigation
done in tumour lysis syndrome?
Serum urate (uric acid).
What are the symptoms
of polycythaemia rubra vera?
- Pruritus (after hot showers)
- Burning sensation of fingers and toes
What are the investigations
done in polycythaemia rubra vera?
- HgB (> 16)
- JAK2 mutation
- ⬇︎ erythropoetin & ferritin
- Hypercellularity
- Thrombocytosis & lymphocytosis
What is the Rx
done in polycythaemia rubra vera?
- Intermittent phlebotomy
- AAS
- Chemotherapy