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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
What is the **Rx** of `DIC`?
**1. Transfusion** - Fresh frozen plasma - PLT **2. Treat the cause**
26
What are the `causes` of **folate deficiency**?
**➜ Dietary** - Malabsorption (coeliac disease, jejunal resection, Chronh's disease); - Poor intake **➜ Drugs** - Methorexate - Trimetoprim - Anticonvulsants - Sulfasalazine
27
What are the `investigations` done in **folate deficiency**?
**◉ FBC** - Anaemia with MCV > 96 - `Serum folate: low`
28
What is the `RX` of **folate deficiency**?
- Folic acid 5mg per day for four months.
29
Describe what is `G6PD deficiency`?
- Inadequate levels of **glutathione reductase** `to protect the red cells from oxidant stress` ➝ **haemolysis**. - X-linked recessive disorder.
30
Describe the **triggers** for a **haemolytic crisis** in `G6PD deficiency`?
- Broad beans (favism) - Primaquine - Nitrofurantoin - Sulfonamides
31
Describe the **symptoms** in `G6PD deficiency`?
- Mostly asymptomatic - Haemolysis - Jaundice
32
Describe the **investigations** in `G6PD deficiency`?
**Blood film** - Heinz bodies and bite cells - Negative coombs test - ↑ LDH - ↓ Haptoglobin
33
Describe the **Rx** for `G6PD deficiency`?
- **Blood transfusion and dialysis (severe cases)** - Folic acid - Avoid triggers - `Splenectomy (last resort)`
34
What are the **types** of `Haemophilia`?
**➔ Haemophilia A** - Factor VIII deficiency **➔ Haemophilia B (Christmas disease)** - Factor IX deficiency
35
What is the **genetic** characteristcs of `Haemophilia`?
X-linked recessive disorder.
36
What are the **symptoms** of `Haemophilia`?
Spontaneous bleeding into: - Joints - Muscles
37
What are the **labs investigation** in `Haemophilia`?
- ↑ APTT **Normal:** - PT - Von Willebrand factor
38
What is the **treatment** of `Haemophilia`?
**Haemophilia A** - Factor VIII infusion - Desmopressin (↑ factor VIII) **Haemophilia B** - Recombinant factor IX
39
What **SHOULD BE AVOIDED** in `Haemophilia`?
**1. NSAIDS** - Gastrointestinal bleeding **2. IM injections** - Haematoma
40
Describe what is **hereditary spherocytosis**?
- Autosomal dominant condition Defect in RBC structural protein ➝ spherocytes ➝ haemolysis in the spleen ➝ anaemia.
41
What are the `symptoms` of **hereditary spherocytosis**?
- Haemolytic anaemia - Jaundice - Splenomegaly - Gallstones
42
What are the `investigations` done in **hereditary spherocytosis**?
**Peripheral smear** - Spherocytes - Howell-Jolly bodies **Others** - Negative directo coombs test - Positive osmotic fragility
43
What will happen if an individual with **hereditary spherocytosis** or with **sickle cell anaemia** if he is infected with `Parvovirus B19`?
Aplastic crisis.
44
What is the `Rx` of **hereditary spherocytosis**?
- Folate supplements - Splenectomy
45
What are the diseases associated with **Parvovirus B19**?
- **Slapped cheek syndrome** - Hereditary spherocytosis - Sickle cell anaemia | B19 is the cause of slapped cheek. ## Footnote - 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
What is **Hodgkin's lymphoma**?
Malignant tumour of the lymphatic system.
47
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
48
What is the `epidemiology` of **Hodgkin's lymphoma**?
- < 25 years - > 55 years
49
What is the `investigation` done in **Hodgkin's lymphoma**?
**Peripheral smear** - Reed - Sternberg cells (multilobulated giant cells)
50
Descrine **NON-Hodgkin's lymphoma**?
Absence of Reed - Sternberg cells.
51
What are the `risk factors` for **Hodgkin's lymphoma**?
- Immunosuppression - Smoking
52
What is one of the types of **`NON`-Hodgkin's lymphoma**?
**Burkitt lymphoma** - Associated with HIV.
53
**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
54
What is the **treatment** of `idiophatic thrombocytopenic purpura`?
- Steroids - Immunoglobulins - Splenectomy
55
What is the **cause** of **`thrombotic thrombocytopenic purpura`**?
Deficiency of **ADAMTS13** - It cleaves the von willebrand factor.
56
What are the **symptoms** of **`thrombotic thrombocytopenic purpura`**? | It's a pentad.
**Pentad** - Fever - Thrombocytopenia - (Microangiopathic) haemolytic anaemia - Renal failure - Neurological abnormalities
57
What is the **labs / investigation** of **`thrombotic thrombocytopenic purpura`**?
- **Blood smear:** Fragmented RBC's - **↓ ADAMTS13 levels** - ↑ creatinine - Microscopic haematuria - ↑ LDH
58
What is the **Rx** of **`thrombotic thrombocytopenic purpura`**?
- Plasma exchange - Steroids **Relapse:** - Rituximab
59
What are the `causes` of **iron deficiency anaemia**?
- ↓ intake - Blood losses from GIT - Menorrhagia
60
What are the `investigation` done in **iron deficiency anaemia**?
**Decreased:** - Ferritin - MCV - HgB - Transferrin saturation **Increased:** - ↑Total iron binding capacity (TIBC)
61
What are the `symptoms` of **iron deficiency anaemia**?
- Koilonychia - Tachychardia - Pallor - Angular stomatitis - Restless legs - Fatigue
62
What is the `Rx` of **iron deficiency anaemia**?
- IV iron - Blood transfusion
63
What is the `association` between **`milk`** and **iron deficiency anaemia**?
Milk decreases iron absorption.
64
**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:** ⟷
65
Describe **Multiple Myeoloma**.
- Cancer of plasma cells; - Infiltrate the bone marrow causing it to replicate the cancerigenous cells.
66
What are the `symptoms` of **Multiple Myeoloma**?
**CRAB** - Hyper`c`alcaemia - `R`enal failure - `A`naemia - `B`one pain
67
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
68
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)
69
What **diseases** are *associated* with `non-blaching rash`?
- Henoch Shonlein purpura - Idiopathic thrombocytopenia - Haemolytic uremic syndrome - Acute leukaemia - Bacterial meningitis
70
Describe what is **Scurby disease**.
Disease resulting from lack of vitamin C.
71
What are the `risk factors` for **Scurby disease**?
- Elderly - Neglected individuals - Chronic alchoolism
72
What are the `symptoms` of **Scurby disease**?
- Spontaneous haematoma - Petechiae - Purpura - Loose teeth - Bleeding gums - Hyperthrophic gums - Lethargy - Pallor
73
How is the `diagnosis` of **Sickle cell disease** made?
- Blood smear: ↑ reticulocytes and sickle cells - HbG electrophosporesis for HbSS
74
How is the `management` of **Sickle cell disease** done?
- Folic acid - Blood transfusions - Oral penicillin prophylaxis - Avoid triggers
75
How is the `pain management` in **Sickle cell disease** made?
**Mild pain** - NSAIDS **Severe pain** - Opioids (`except pethidine`) ## Footnote Pethidine leads to accumulation of metabolites in sickle cell and causes neurotoxicity.
76
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 bleed** `INR > 5` - Stop warfarin - Give Vit K IV **No bleed** `INR > 8` - Stop warfarin - Give Vit K oral `INR > 8` - Stop warfarin
77
What are the **types** of `thalassaemias`?
- Alpha thalassemia: die in utero - **Beta thalassemia**
78
What are the `symptoms` of **Beta thalassemia major**?
- Frontal bossing - Haemolytic anaemia - Hepatosplenomegaly - Iron overload (due to repeated transfusions)
79
What are the `investigations` done in **Beta thalassemia major**?
- ↑ serum iron - ↑ ferritin saturation - ↑ ferritin - Microcytic hypochromic anaemia - ↑ HbA2 on electrosphoresis
80
What is the `treatment` of **Beta thalassemia major**?
- Life long blood transfusion - Iron chelation (prevents overload)
81
# Von Willebrand disease What is the **function** of `Von Willebrand factor`?
- Facilitates PLT adhesion to endothelial cells - Carrier molecule for factor VIII
82
Describe `Von Willebrand disease`.
Abnormal function or deficiency of Von Willebrand factor. - PLT can't form plug - Factor VIII degradates
83
What are the **symptoms** of `Von Willebrand disease`?
- Epistaxis - Mucosal bleeding - Easy bruising - Menorrhagia
84
What are the **investigations** done in `Von Willebrand disease`?
- Prolongued aPTT - INR & PT: Normal - PLT: normal
85
What is the **Rx** of `Von Willebrand disease`?
- Tranexamic acid - Desmopressin (releases vWF from endothelium)
86
Describe what is **tumour lysis syndrome**.
Massive cell death in chemotheraphy.
87
Describe the `presentation` of **tumour lysis syndrome**.
- **Hyperuricemia** - Hyperkalaemia - Hyperphosphatemia - Hypocalcemia
88
What is the `Rx` of **tumour lysis syndrome**?
- IV fluids - Diuretics - Rasburicase - Kidney failure: haemodialysis - **Allopurinol (prevention)**
89
What is the `investigation` done in **tumour lysis syndrome**?
Serum urate (uric acid).
90
What are the `symptoms` of **polycythaemia rubra vera**?
- Pruritus (after hot showers) - Burning sensation of fingers and toes
91
What are the `investigations` done in **polycythaemia rubra vera**?
- HgB (> 16) - **JAK2 mutation** - **⬇︎ erythropoetin & ferritin** - **Hypercellularity** - Thrombocytosis & lymphocytosis
92
What is the `Rx` done in **polycythaemia rubra vera**?
- Intermittent phlebotomy - AAS - Chemotherapy

PLAB 1 August 2024 (32 decks)

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