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PLAB 1 August 2024 > Haematology > Flashcards

Haematology Flashcards

1
Q

Which are the ACUTE leukaemia types / classifications?

A
  • Acute lymphoblastic leukaemia (ALL)
  • Acute myeloid leukaemia (AML)
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2
Q

What is the epidemiology of ALL?

A

Children.

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3
Q

What are the symptoms of ALL?

A
  • Fatigue (anaemia)
  • Bleeding, petechia, purpura, ecchymoses (trombocytopenia)
  • Recurrent infections (low or abnormal WBC’s)
  • LUQ tenderness & early satiety (splenomegaly).
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4
Q

Diagnosis of ALL (acute lymphoblastic leukaemia)

A

► 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.

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5
Q

What is the epidemiology of AML?

A

Mostly elderly.

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6
Q

What are the symptoms of AML?

Acute myeloid leukaemia

A

⬇︎ RBC
- Anaemia
- Fatigue

⬆︎ WBC
- Fever
- Leukocytosis / leukopenia
- Gingivitis

⬇︎ PLT
- Bleeding
- Hepatosplenomegaly

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7
Q

Diagnosis of AML ?

A

► Most initial ⟶ FBC (abnormal)
► Most appropriate ⟶Peripheral smear: Auer rods
► Most definitive test ⟶Bone marrow biopsy: Myeloblasts

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8
Q

Where are the following absorbed in the body?
- B12
- Folate

A

B12
-Terminal ileum

Folate
- Jejunum / duodenum

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9
Q

What are the causes of Vitamin B12 defficiency?

A
  • Vegan diet
  • Gastrectomy
  • Pernicious anaemia
  • Ileal disease
  • Chrohn’s disease
  • Malabsorption diseases
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10
Q

What are the symptoms of Vitamin B12 defficiency?

A
  • Macrocytic anaemia
  • Pins and needles on the limbs
  • Cognitive changes
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11
Q

What is the Rx of Vitamin B12 defficiency?

A

Hidroxycobalamin IM.

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12
Q

DDx of peripheral smear in leukaemias.

A

ALL
- Lymphoblasts

AML
- Myeloblast
- Auer rods

CLL
- Small mature lymphocytes
- Smudge cells

CML
- Mature myeloblasts
- ⬆︎ Basophils

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13
Q

What are the symptoms of CLL?

Chronic lymphocytic leukaemia

A
  • Leukocytosis
  • Enlarged lymph nodes
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14
Q

Diagnosis of CLL

A

Peripheral blood smear
-Smudge cells

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15
Q

What is the epidemiology of CML?

Chronic myelocytic leukaemia

A

Elderly.

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16
Q

What are the symptoms of CML?

A
  • Leukocytosis
  • Massive splenomegaly
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17
Q

Diagnosis of CML

A

Peripheral blood smear
- All stages of maturation seen
- Basophils increased

Genetics
- Philadelphia chromossome

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18
Q

DDx between:
- Von Willebrand
- Haemophilia
- Disseminated intravascular coagulation

A

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.
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19
Q

DDx between:
- Vit B12 deficiency
- Folate deficiency

A

Vit B12 deficiency
- Vegans
- Found in meats

Folate deficiency
- Meat eaters
- Found in vegetables

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20
Q

DDx between:
- Direct coombs test
- Indirect coombs test

A

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

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21
Q

Describe Disseminated intravascular coagulation.

A
  • Widespread activation of the coagulation cascade;
  • Results in ⬇︎ clotting factors;
  • Blood clots in different organs;
  • Bleeding.
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22
Q

What are the causes of DIC?

A
  • Sepsis
  • Major trauma
  • Complications of pregnancy
  • Cancer
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23
Q

What are the symptoms of DIC?

A
  • Bleeding
  • Purpura
  • Petechiae
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24
Q

What are the investigations / LABS of DIC?

A

Increased:
- PT
- aPTT
- INR
- D-Dimer

Decreased:
- PLT
- Fibrinogen

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25
Q

What is the Rx of DIC?

A

1. Transfusion
- Fresh frozen plasma
- PLT

2. Treat the cause

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26
Q

What are the causes of folate deficiency?

A

➜ Dietary
- Malabsorption (coeliac disease, jejunal resection, Chronh’s disease);
- Poor intake

➜ Drugs
- Methorexate
- Trimetoprim
- Anticonvulsants
- Sulfasalazine

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27
Q

What are the investigations done in folate deficiency?

A

◉ FBC
- Anaemia with MCV > 96
- Serum folate: low

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28
Q

What is the RX of folate deficiency?

A
  • Folic acid 5mg per day for four months.
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29
Q

Describe what is G6PD deficiency?

A
  • Inadequate levels of glutathione reductase to protect the red cells from oxidant stress ➝ haemolysis.
  • X-linked recessive disorder.
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30
Q

Describe the triggers for a haemolytic crisis in G6PD deficiency?

A
  • Broad beans (favism)
  • Primaquine
  • Nitrofurantoin
  • Sulfonamides
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31
Q

Describe the symptoms in G6PD deficiency?

A
  • Mostly asymptomatic
  • Haemolysis
  • Jaundice
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32
Q

Describe the investigations in G6PD deficiency?

A

Blood film
- Heinz bodies and bite cells
- Negative coombs test
- ↑ LDH
- ↓ Haptoglobin

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33
Q

Describe the Rx for G6PD deficiency?

A
  • Blood transfusion and dialysis (severe cases)
  • Folic acid
  • Avoid triggers
  • Splenectomy (last resort)
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34
Q

What are the types of Haemophilia?

A

➔ Haemophilia A
- Factor VIII deficiency

➔ Haemophilia B (Christmas disease)
- Factor IX deficiency

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35
Q

What is the genetic characteristcs of Haemophilia?

A

X-linked recessive disorder.

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36
Q

What are the symptoms of Haemophilia?

A

Spontaneous bleeding into:
- Joints
- Muscles

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37
Q

What are the labs investigation in Haemophilia?

A
  • ↑ APTT

Normal:
- PT
- Von Willebrand factor

38
Q

What is the treatment of Haemophilia?

A

Haemophilia A
- Factor VIII infusion
- Desmopressin (↑ factor VIII)

Haemophilia B
- Recombinant factor IX

39
Q

What SHOULD BE AVOIDED in Haemophilia?

A

1. NSAIDS
- Gastrointestinal bleeding
2. IM injections
- Haematoma

40
Q

Describe what is hereditary spherocytosis?

A
  • Autosomal dominant condition
    Defect in RBC structural protein ➝ spherocytes ➝ haemolysis in the spleen ➝ anaemia.
41
Q

What are the symptoms of hereditary spherocytosis?

A
  • Haemolytic anaemia
  • Jaundice
  • Splenomegaly
  • Gallstones
42
Q

What are the investigations done in hereditary spherocytosis?

A

Peripheral smear
- Spherocytes
- Howell-Jolly bodies

Others
- Negative directo coombs test
- Positive osmotic fragility

43
Q

What will happen if an individual with hereditary spherocytosis or with sickle cell anaemia if he is infected with Parvovirus B19?

A

Aplastic crisis.

44
Q

What is the Rx of hereditary spherocytosis?

A
  • Folate supplements
  • Splenectomy
45
Q

What are the diseases associated with Parvovirus B19?

A
  • 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.
46
Q

What is Hodgkin’s lymphoma?

A

Malignant tumour of the lymphatic system.

47
Q

What are the symptoms of Hodgkin’s lymphoma?

A

General
- Painless lymphadenopathy
- Splenomegaly
- Weight loss
- Hepatomegaly
- B symptoms

B symptoms
- Weight loss
- Night sweats
- Fever > 38
- Fatigue

48
Q

What is the epidemiology of Hodgkin’s lymphoma?

A
  • < 25 years
  • > 55 years
49
Q

What is the investigation done in Hodgkin’s lymphoma?

A

Peripheral smear
- Reed - Sternberg cells (multilobulated giant cells)

50
Q

Descrine NON-Hodgkin’s lymphoma?

A

Absence of Reed - Sternberg cells.

51
Q

What are the risk factors for Hodgkin’s lymphoma?

A
  • Immunosuppression
  • Smoking
52
Q

What is one of the types of NON-Hodgkin’s lymphoma?

A

Burkitt lymphoma
- Associated with HIV.

53
Q

DDx between:
- Children idiophatic thrombocytopenic purpura
- Adults idiophatic thrombocytopenic purpura

A

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

54
Q

What is the treatment of idiophatic thrombocytopenic purpura?

A
  • Steroids
  • Immunoglobulins
  • Splenectomy
55
Q

What is the cause of thrombotic thrombocytopenic purpura?

A

Deficiency of ADAMTS13
- It cleaves the von willebrand factor.

56
Q

What are the symptoms of thrombotic thrombocytopenic purpura?

It’s a pentad.

A

Pentad
- Fever
- Thrombocytopenia
- (Microangiopathic) haemolytic anaemia
- Renal failure
- Neurological abnormalities

57
Q

What is the labs / investigation of thrombotic thrombocytopenic purpura?

A
  • Blood smear: Fragmented RBC’s
  • ↓ ADAMTS13 levels
  • ↑ creatinine
  • Microscopic haematuria
  • ↑ LDH
58
Q

What is the Rx of thrombotic thrombocytopenic purpura?

A
  • Plasma exchange
  • Steroids

Relapse:
- Rituximab

59
Q

What are the causes of iron deficiency anaemia?

A
  • ↓ intake
  • Blood losses from GIT
  • Menorrhagia
60
Q

What are the investigation done in iron deficiency anaemia?

A

Decreased:
- Ferritin
- MCV
- HgB
- Transferrin saturation

Increased:
- ↑Total iron binding capacity (TIBC)

61
Q

What are the symptoms of iron deficiency anaemia?

A
  • Koilonychia
  • Tachychardia
  • Pallor
  • Angular stomatitis
  • Restless legs
  • Fatigue
62
Q

What is the Rx of iron deficiency anaemia?

A
  • IV iron
  • Blood transfusion
63
Q

What is the association between milk and iron deficiency anaemia?

A

Milk decreases iron absorption.

64
Q

DDx between:
- Iron deficiency anaemia
- Anaemia of chronic disease
- Thalassaemia trait

A

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: ⟷

65
Q

Describe Multiple Myeoloma.

A
  • Cancer of plasma cells;
  • Infiltrate the bone marrow causing it to replicate the cancerigenous cells.
66
Q

What are the symptoms of Multiple Myeoloma?

A

CRAB
- Hypercalcaemia
- Renal failure
- Anaemia
- Bone pain

67
Q

What are the investigations done in Multiple Myeoloma?

A
  • 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
68
Q

What are the step by step investigations done in Multiple Myeoloma?

A

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)

69
Q

What diseases are associated with non-blaching rash?

A
  • Henoch Shonlein purpura
  • Idiopathic thrombocytopenia
  • Haemolytic uremic syndrome
  • Acute leukaemia
  • Bacterial meningitis
70
Q

Describe what is Scurby disease.

A

Disease resulting from lack of vitamin C.

71
Q

What are the risk factors for Scurby disease?

A
  • Elderly
  • Neglected individuals
  • Chronic alchoolism
72
Q

What are the symptoms of Scurby disease?

A
  • Spontaneous haematoma
  • Petechiae
  • Purpura
  • Loose teeth
  • Bleeding gums
  • Hyperthrophic gums
  • Lethargy
  • Pallor
73
Q

How is the diagnosis of Sickle cell disease made?

A
  • Blood smear: ↑ reticulocytes and sickle cells
  • HbG electrophosporesis for HbSS
74
Q

How is the management of Sickle cell disease done?

A
  • Folic acid
  • Blood transfusions
  • Oral penicillin prophylaxis
  • Avoid triggers
75
Q

How is the pain management in Sickle cell disease made?

A

Mild pain
- NSAIDS

Severe pain
- Opioids (except pethidine)

Pethidine leads to accumulation of metabolites in sickle cell and causes neurotoxicity.

76
Q

What is the management of warfarin with high INR and bleeding?

A

Massive bleeding
- Stop warfarin
- Administer phrotrombin complex concentrate
- Administer Vitamin K IV
- Fresh frozen plasma (if no PCC)

Minor bleed
INR > 5
- Stop warfarin
- Give Vit K IV

No bleed
INR > 8
- Stop warfarin
- Give Vit K oral
INR > 8
- Stop warfarin

77
Q

What are the types of thalassaemias?

A
  • Alpha thalassemia: die in utero
  • Beta thalassemia
78
Q

What are the symptoms of Beta thalassemia major?

A
  • Frontal bossing
  • Haemolytic anaemia
  • Hepatosplenomegaly
  • Iron overload (due to repeated transfusions)
79
Q

What are the investigations done in Beta thalassemia major?

A
  • ↑ serum iron
  • ↑ ferritin saturation
  • ↑ ferritin
  • Microcytic hypochromic anaemia
  • ↑ HbA2 on electrosphoresis
80
Q

What is the treatment of Beta thalassemia major?

A
  • Life long blood transfusion
  • Iron chelation (prevents overload)
81
Q

Von Willebrand disease

What is the function of Von Willebrand factor?

A
  • Facilitates PLT adhesion to endothelial cells
  • Carrier molecule for factor VIII
82
Q

Describe Von Willebrand disease.

A

Abnormal function or deficiency of Von Willebrand factor.
- PLT can’t form plug
- Factor VIII degradates

83
Q

What are the symptoms of Von Willebrand disease?

A
  • Epistaxis
  • Mucosal bleeding
  • Easy bruising
  • Menorrhagia
84
Q

What are the investigations done in Von Willebrand disease?

A
  • Prolongued aPTT
  • INR & PT: Normal
  • PLT: normal
85
Q

What is the Rx of Von Willebrand disease?

A
  • Tranexamic acid
  • Desmopressin (releases vWF from endothelium)
86
Q

Describe what is tumour lysis syndrome.

A

Massive cell death in chemotheraphy.

87
Q

Describe the presentation of tumour lysis syndrome.

A
  • Hyperuricemia
  • Hyperkalaemia
  • Hyperphosphatemia
  • Hypocalcemia
88
Q

What is the Rx of tumour lysis syndrome?

A
  • IV fluids
  • Diuretics
  • Rasburicase
  • Kidney failure: haemodialysis
  • Allopurinol (prevention)
89
Q

What is the investigation done in tumour lysis syndrome?

A

Serum urate (uric acid).

90
Q

What are the symptoms of polycythaemia rubra vera?

A
  • Pruritus (after hot showers)
  • Burning sensation of fingers and toes
91
Q

What are the investigations done in polycythaemia rubra vera?

A
  • HgB (> 16)
  • JAK2 mutation
  • ⬇︎ erythropoetin & ferritin
  • Hypercellularity
  • Thrombocytosis & lymphocytosis
92
Q

What is the Rx done in polycythaemia rubra vera?

A
  • Intermittent phlebotomy
  • AAS
  • Chemotherapy

PLAB 1 August 2024 (32 decks)

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