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Passmed questions Haem Flashcards

1
Q

What subtype of Hodgkin’s lymphoma has the most favourable prognosis?

A

Classical lymphocyte predominant

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2
Q

Classical histological section of Hodgkin’s lymphoma?

A

Reed Sternberg cells (+ lymph node pain on alcohol consumption)

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3
Q

Translocation t(8;14) condition?

A

Burkitt’s lymphoma

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4
Q

Burkitt’s lymphoma translocation?

A

t(8;14)

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5
Q

Translocation t(9;22) condition?

A

CML, BCR-ABL gene

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6
Q

CML translocation?

A

t(9;22), BCR-ABL gene

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7
Q

Translocation t(11;14) condition?

A

Mantle cell lymphoma, BCL-1

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8
Q

Mantle cell lymphoma translocation?

A

t(11;14), deregulation of the cyclin D1 (BCL-1) gene

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9
Q

Translocation t(14;18) condition?

A

Follicular lymphoma, BCL-2

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10
Q

Follicular lymphoma translocation?

A

t(14;18), BCL-2

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11
Q

Translocation t(15;17) condition?

A

Acute promyelocytic lymphoma

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12
Q

Acute promyelocytic lymphoma translocation?

A

t(15;17)

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13
Q

How does methotrexate produce a megaloblastic anaemia?

A

It can produce a megaloblastic microcytic anaemia secondary to folate deficiency

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14
Q

What is the most common adverse event of transfusion packed red blood cells?

A

Pyrexia

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15
Q

What is the most common adverse event of FFP infusion?

A

Urticaria

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16
Q

Types of blood transfusion complications

A

Immunological: acute haemolytic, non-haemolytic febrile, allergic/anaphylaxis

Infective

Transfusion-related acute lung injury (TRALI)

Transfusion-associated circulatory overload (TACO)

Other: hyperkalaemia, iron overload, clotting

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17
Q

Non-haemolytic febrile reaction

A

Caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage

RBC transfusion (1-2%), platelet transfusion (10-30%)

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18
Q

Minor allergic reaction

A

Caused by foreign plasma proteins

Symptoms include pruritus and urticaria - give antihistamine

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19
Q

Anaphylaxis

A

Caused by patients with IgA deficiency who have anti-IgA antibodies (these are usually IgE antibodies)

Symptoms include hypotension, dyspnoea, wheezing, angioedema

ABC support and give IM adrenaline

20
Q

Acute haemolytic reaction

A

Caused by ABO-incompatible blood e.g. secondary to human error. Usually host IgM antibody-mediated destruction of incompatible RBCs

Symptoms include fever. abdominal pain, hypotension

Send blood for direct Coombs test, repeat typing and cross-matching + fluid resuscitation

21
Q

Transfusion-associated circulatory overload (TACO)

A

Caused by excessive rate of transfusion, pre-existing heart failure (fluid overload)

Symptoms include pulmonary oedema, hypertension

Consider intravenous loop diuretic (e.g. furosemide) and oxygen

22
Q

Transfusion-related acute lung injury (TRALI)

A

Caused by non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood

Symptoms include hypoxia, pulmonary infiltrates on chest x-ray, fever, hypotension

Stop transfusion, give oxygen and supportive care

23
Q

Which clotting factors does heparin affect?

A

Heparin prevents the activation of factors 2,9,10,11 (acceleration of antithrombin against these factors)

24
Q

Which clotting factors does warfarin affect?

A

Warfarin affects the synthesis of factors 2,7,9,10 (vitamin K dependent)

25
Which factors does DIC affect?
Factors 1,2,5,8,11
26
Which factors does liver disease affect?
Factors 1,2,5,7,9,10,11
27
APTT, PT and bleeding time in haemophilia?
APTT: increased PT: normal Bleeding time: normal
28
APTT, PT and bleeding time in von Willebrand's disease?
APTT: increased PT: normal Bleeding time: increased
29
APTT, PT and bleeding time in vitamin K deficiency?
APTT: increased PT: increased Bleeding time: normal
30
What is the standard recommended chemotherapy regime in non-hodgkin's lymphoma?
R-CHOP Rituximab (in certain patients - cardio toxicity; infections Cyclophosphamide - nausea and vomiting; bone marrow suppression; hemorrhagic cystitis Hydroxydaunorubicin - dilated cardiomyopathy with progression to heart failure (and high mortality) Oncovin (cinvristine) - peripheral neuropathy with tingling and numbness; neuropathic pain Prednisolone - proximal neuropathy
31
Pathophysiology of hypercalcaemia in multiple myeloma?
Osteolyisis through release of cytokines by myeloma cells which increase osteoclast activity. This causes bone pain and pathological osteolytic lesions
32
What cellular types/features do you see in sarcoidosis?
T-lymphocytes, macrophages, asteroid bodies, B-lymphocytes
33
Pathophysiology of G6PD deficiency?
X-linked recessive condition causing RBC enzyme defect ↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress
34
What is sideroblastic anaemia
Red cells fail to completely form haem, whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus called a ring sideroblast. It may be congenital or acquired.
35
Profile of sideroblastic anaemia?
Hypochromic microcytic anaemia High ferritin, iron and transferrin saturation Basophilic stippling of RBCs on blood film Ringed sideroblasts (Prussian blue staining) in bone marrow
36
Causes of sideroblastic anaemia
Congenital cause: Delta-aminolevulinate synthase-2 deficiency Acquired causes Myelodysplasia Alcohol Lead Anti-TB medications
37
Complications of tumour lysis syndrome
Hyperkalaemia Hyperphosphataemia Hypocalcaemia - secondary to chelation of free calcium to phosphate Hyperuricaemia - secondary to DNA metabolism Acute kidney injury - secondary to hyperuricaemia (prophylaxis using allopurinol)
38
What is factor V Leiden?
It is an inherited thrombophilia where there is a mutation to the factor V gene which means protein C (anticoagulant) can no longer bind to it normally. This protein C resistance leads to inefficient inactivation (10x more slowly) of factor V, resulting in overproduction of thrombin and excess clotting.
39
How does antiphospholipid syndrome increase blood clotting?
Auto-antibodies bind to plasma membranes and platelets which increase clotting. They also inhibit the function of protein C and protein S.
40
Side effects of phenytoin (for seizures)
It is an anti-folate drug and can cause folate deficiency, resulting in macrocytic anaemia
41
Normoblastic causes of macrocytic anaemia?
Alcohol Liver disease Hypothyroidism Pregnancy Reticulocytosis Myelodysplasia Drugs: cytotoxics
42
Megaloblastic causes of macrocytic anaemia?
Vitamin B12 (meat and dairy) deficiency Folate (vegetables, nuts) deficiency e.g. drugs like methotrexate, phenytoin
43
What does Howell-Jolly bodies in a peripheral smear of a sickle cell patient indicate?
Autosplenectomy (fibrosis secondary to splenic infarctions). This hyposplenism means the spleen is incapable of removing nucleated RBCs from circulation, so they appear with clusters of basophilic nuclear remnants, also known as Howell jolly bodies
44
How does parvovirus 19 (erythrovirus 19) affect blood cells?
Usually following haemolytic and affects red blood cell precursors in the bone marrow --> reduced erythropoiesis. This causes anaemia with a fall in reticulocyte count
45
What is splenic sequestration (sickle cell anaemia)?
It is the trapping of RBCs in the spleen, causing massive painful enlargement. This is followed with a dramatic fall in Hb, with potential for hypovolaemic shock
46
What are the different phenotypes of alpha thalassaemia?
Number of missing alleles Genotype Condition: 1 (aa/a-) Silent carrier (alpha(+) heterozygous) 2 (aa/--) Alpha thalassaemia trait: alpha(0) heterozygous 2 (a-/a-) Alpha thalassaemia trait: alpha(+) homozygous 3 (a-/--) Haemoglobin H disease 4 (--/--) Alpha thalassaemia major (usually causing hydrops foetalis) also Hb Parts

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