haematology

Question 1

Types of leukemia and how they look like on blood film

Answer 1

Lymphoid cells: NK cells, b lymphocytes, t lymphocytes, plasma cells
Myeloid cells: myelobasts –> neutrophils, basophils, eosinophils, monocytes/macrophages

ALL:
AML:
CML: mature and immature neutrophils
CLL:

Question 2

translocation for burkitts lyphoma

Answer 2

t (8:14)

Question 3

translocation for CML

Answer 3

t(9:22)

BCR-ABL

Question 4

What is GVHD

Answer 4

Acute <6 months
Chronic >6 months
multi-system: skin, liver, gut

skin - rash on soles and palms
Liver - mildly derranged LFTS
gut - diarrhoea, abnormal colour

Management: good nutrition, weight maintenance and immunosuppression with methylprednisone and cyclosporin.
Tacrolim

Question 5

What is transfusion GVHD

Answer 5

post BMT, host cannot mount an immune response
When lymphocytes in donor bood attack host tissues

Clinical features: identical to GVHD
typically occurs 14 days after transfusion

Management: avoid tranfusions, irradiated blood (kills lymphocytes)

Question 6

CMV Infection post transplant

Answer 6

Diarrhoea

Derranged LFTs

Myelosuppression

Question 7

transfusion hypersensitity reaction

Answer 7

erythema, urticario, bronchospasma (wheeze/crackles/chest tightness), fever

MX: adrenaline, hydrocortison, chlorphenamine

Question 8

Signs of TTP

Answer 8

MAHA
Thrombocytopenia
Neuro dysfunction
renal impairment
fever

ADAMTS13 deficiency

Question 9

Management of TTP

Answer 9

Plasmapharesis (exhange) and FFP

Question 10

Whats is waldenstrom macroglobulinemia

Answer 10

lyphoplasmacytuc lymphoma - IgM paraprotein

COmplications: hyperviscosity syndrome

Question 11

test and treatment of hyperviscosity syndrome

Answer 11

plasma viscosity

plasmapharesis to remov IgM

Question 12

CVID

Answer 12

reduced IgG and IgA
1. Recurrent chest infections
2, Autoimmune disease
3. Lymphoid hyperplasia/granulomatous disease
4. Malignancy

Question 13

leukoerythroblastic reactions

Answer 13

peripheral blood contains immature white cells and nucleated red cells irrespective of the total white cell count and circulating blasts may be seen.

Question 14

Managwmwnt of bone pain

Answer 14

radiotherapy

Question 15

what is PNH

Answer 15

Erthrocytes are sensitive to lysis by complement
Panyctopenia
Ptoyhrobotic
Dark urine in the morning

Question 16

CLL and cytometry

Answer 16

Cytometry is gold standard
Picks up monolclonal B cells proliferation (CD19/5 co expressing, CD23 positive, light chain restricted B cell population) is diagnostic of CLL.

Question 17

Neutropenic sepsis management

Answer 17

anti pseudomonal (piptaz) and gent

antifungal if no improvement

Question 18

treatment for post splenectomy and chronic ITP with bleeding

Answer 18

immunosupression agent (not steroid)

Question 19

management of iron overload in chronic anemia from multiple transufsions (secondary haemochromatosis)

Answer 19

oral iron chelation with deferasirox is the first line gold standard treatment for iron chelation in iron overload related to repeated transfusions in patients with chronic anaemias.

Question 20

symptoms of hyperviscosity syndrome

Answer 20

dizziness
tinnitus
headaches
blurred vision, and
pruritus.

Question 21

causes of Polycythemia ruvra vera

Answer 21

A classic symptom of polycythemia vera is pruritus or itching, particularly after exposure to warm water (such as when taking a bath), which may be due to abnormal histamine release1 or prostaglandin production2.

Signs include:

Various ophthalmological changes (for example, dilated retinal veins)
Neurological findings, and
Facial plethora ('ruddy' appearance).

Secondary causes of polycythaemia include erythropoietin-secreting tumours and causes of chronic hypoxia

Erythropoietin-secreting tumours:

Renal cell carcinoma
Hepatocellular carcinoma
Haemangioblastoma
Uterine fibroids.
Chronic hypoxia:

COPD
Right-to-left cardiac shunts
Sleep apnoea
High altitude
Chronic carbon monoxide poisoning (including heavy smoking).

Question 22

TRALI

Answer 22

Fever

Hypoxia

Question 23

von Willebrand’s disease

Answer 23

von Willebrand’s disease is associated with mucosal bleeding and abnormal bleeding time.

Question 24

HHT

Answer 24

recent headaches, recurrent epistaxis, and an episode of haemoptysis with probable pulmonary haemorrhages on his chest XR. These findings are highly suggestive of hereditary haemorrhagic telangiectasia (HHT).

Other clues are punctiform oral lesions, a sister with a history of gastrointestinal bleeding in absence of abnormal clotting or thrombocytopenia.

There are two genetic types associated with mutations involving the endoglin gene on chromosome 9q and activin receptor-like kinase 1 located on 12q. HHT typically presents with epistaxis, and arteriovenous malformations of the brain and lung.

Question 25

PNH

Answer 25

Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal stem cell disorder resulting in deficiency of GPI-anchored proteins and intravascular haemolysis, cytopenias and thrombosis.
Ham test for lysis

Question 26

management of painful sickle cell crisis

Answer 26

Painful crises in sickle cell anaemia should be managed aggressively with hydration and analgesia, typically necessitating opioid analgesia in view of the severity of the pain involved. NSAIDs do not usually provide effective analgesia on their own in sickle cell painful crises,

Question 27

management of warm-autoimmune hemolytic anemia

Answer 27

Warm antibody auto-immune haemolytic anaemia is highly responsive to corticosteroids and these are the initial therapy of choice.