haem Flashcards

1
Q

Waldenstroms triad

A

B-cell lymphoma characterised by organomegaly
pancytopenia
cold autoimmune haemolytic anaemia

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2
Q

Waldenstroms pathology

A

B cells producing excess IGM

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3
Q

Symptoms associated with Waldenstroms

A

Hyperviscosity Symptoms
(visual disturbance, headache)

Plus: anaemia, thrombocytopaenia, raised white cell count, organomegaly with lymphadenopathy
elevated IgM

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4
Q

Myeloma most common IG band

A

IG-A

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5
Q

Organomegaly in myeloma?

A

No

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6
Q

MGUS has no CRAB symptoms- what are CRAB symptoms

A

hyperCalcaemia
Renal failure
Anaemia
Bone pain.

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7
Q

IG- what is pentameric

A

IG-M

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8
Q

Philadelphia chromosome - prognosis

A

GOOD in CML
BAD in AML/ALL

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9
Q

Cyclophosphamide side effect

A

Haemorrhagic cystitis, myelosuppression, transitional cell carcinoma

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10
Q

Bleomycin side effects

A

Lung fibrosis

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11
Q

Anthracyclines (e.g doxorubicin) side effects

A

Cardiomyopathy

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12
Q

Methotrexate side effects

A

Myelosuppression, mucositis, liver fibrosis, lung fibrosis

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13
Q

Fluorouracil (5-FU) side effects

A

Myelosuppression, mucositis, dermatitis

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14
Q

6-mercaptopurine side effects

A

Myelosuppression

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15
Q

Cytarabine side effects

A

Myelosuppression, ataxia

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16
Q

Vincristine, vinblastine side effects

A

Vincristine: Peripheral neuropathy (reversible) , paralytic ileus
Vinblastine: myelosuppression

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17
Q

Docetaxel side effects

A

Neutropaenia

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18
Q

Irinotecan side effects

A

Myelosupression

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19
Q

Cisplatin side effects

A

Ototoxicity, peripheral neuropathy, hypomagnesaemia

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20
Q

Hydroxyurea (hydroxycarbamide) side effects

A

Myelosuppression

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21
Q

Interferon-gamma is produced by which cells

A

primarily by natural killer cells and T helper cells

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22
Q

Interferon-beta is produced by which cells

A

Fibroblasts

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23
Q

Interferon-alpha is produced by which cells

A

Interferon-alpha is produced by leucocytes

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24
Q

Monocytes are white blood cells that give rise to

A

macrophages and dendritic cells in the immune system. They produce cytokines such as IL-6 in response to infections and tissue injuries.

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25
Q

Smudge/smear cells

A

CLL

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26
Q

Essential thrombocytosis , treatment of choice

A

Hydroxyurea

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27
Q

Extravascular haemolysis

A

thalassaemia

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28
Q

Siderotic granules

A

hyposplenism

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29
Q

Auer rods

A

apml

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30
Q

Target cells

A

liver disease
sickle-cell anaemia
Fe deficiency

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31
Q

Heinz bodies

A

G6PD

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32
Q

anemia casued by hypothyroidism

A

macrocytic

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33
Q

‘tear-drop’ poikilocytes

A

myelofibrosis

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34
Q

Intravascular haemolysis on blood film

A

schistocytes

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35
Q

Spherocytes

A

AIHA

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36
Q

Basophilic stippling

A

Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia

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37
Q

Howell-Jolly bodies

A

Hyposplenism

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38
Q

Schistocytes (‘helmet cells’)

A

Intravascular haemolysis
Mechanical heart valve
Disseminated intravascular coagulation

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39
Q

Burr cells (echinocytes)

A

Uraemia
Pyruvate kinase deficiency

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40
Q

Acanthocytes

A

Abetalipoproteinemia

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41
Q

Splenomegaly causes

A

Massive splenomegaly
myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher’s syndrome

Other causes (as above plus)
portal hypertension e.g. secondary to cirrhosis
lymphoproliferative disease e.g. CLL, Hodgkin’s
haemolytic anaemia
infection: hepatitis, glandular fever
infective endocarditis
sickle-cell*, thalassaemia
rheumatoid arthritis (Felty’s syndrome)

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42
Q

platelet transfusion in surgery targets

A

Platelet transfusion for thrombocytopenia before surgery/ an invasive procedure. Aim for plt levels of:
> 50×109/L for most patients
50-75×109/L if high risk of bleeding
>100×109/L if surgery at critical site

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43
Q

epidemiology of hodgkins/non-hodgkins

A

Non-Hodgkin’s lymphoma is much more common than Hodgkin’s lymphoma

44
Q

Symptoms of NHL

A

Symptoms
Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
Constitutional/B symptoms (fever, weight loss, night sweats, lethargy)
Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)

45
Q

CLL complications

A

Chronic lymphocytic leukaemia: complications
Complications
anaemia
hypogammaglobulinaemia leading to recurrent infections
warm autoimmune haemolytic anaemia in 10-15% of patients
transformation to high-grade lymphoma (Richter’s transformation)

46
Q

Richters transformation in CLL>?

A

Richter’s transformation

Ritcher’s transformation occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma. Patients often become unwell very suddenly.

Ritcher’s transformation is indicated by one of the following symptoms:
lymph node swelling
fever without infection
weight loss
night sweats
nausea
abdominal pain

47
Q

Percentages of iron in the body?

A

total body iron = 4g
haemoglobin = 70%
ferritin and haemosiderin = 25%
myoglobin = 4%
plasma iron = 0.1%

48
Q

iron is stored as

A

ferritin

49
Q

iron is transported by

A

carried in plasma as Fe3+ bound to transferrin

50
Q

DIC tests suggestive of

A

Diagnosis of DIC involves a combination of laboratory tests and clinical evaluation. Laboratory findings suggestive of DIC include a low platelet count, elevated D-dimer concentration, decreased fibrinogen concentration, and prolongation of clotting times such as prothrombin time (PT).

51
Q

warfarin PT/APTT/Fibrinogen/Bleeding time/PLt

A

PT prolonged - normal everything else

52
Q

Asprin PT/APTT/Fibrinogen/Bleeding time/PLt

A

Prolonged bleeding time normal everythign else

53
Q

DIC PT/APTT/Fibrinogen/Bleeding time/PLt

A

Prolonged PT/APPT low fibrinogen low platlets

54
Q

Aplastic crisis in sickle cell

A

Following parvovirus infection - pancytopenia

55
Q

Acute chest syndrome in sickle cell

A

pain/cough/wheeze/sob
CT pulm infiltrates

56
Q

Sequestration crisis in sickle cell

A

presents with abdominal pain, signs of haemodynamic compromise and hepatomegaly/splenomegaly. Pooling of blood in the spleen occurs, leading to severe anaemia and haemodynamic collapse.

57
Q

Ann arbor staging I

A

Stage I - involves a single regional lymph node

58
Q

Ann arbor staging II

A

Stage II - involves two or more lymph nodes on one side of the diaphragm

59
Q

Ann arbor staging III

A

Stage III of the Ann-Arbor clinical staging of lymphomas involve lymph nodes on both sides of the diaphragm

60
Q

Ann arbor staging IV

A

Stage IV - distant spread involving one or more extra lymphatic organs

61
Q

Ann Arbor A/B

A

A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)

62
Q

Lymphoma treatement

A

ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine): considered the standard regime

BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone): alternative regime with better remission rates but higher toxicity

63
Q

most common inherited thrombophilia?

A

Activated protein C resistance (Factor V Leiden) is the most common inherited thrombophilia

64
Q

Bleomycin side effect

A

Lung fibrosis

65
Q

Doxyrubcin side effect

A

Cardiomyopathy

66
Q

Cyclophosphamide side effect

A

haemoraggic cystitis
TCC

67
Q

Vincristine side effect

A

peripheral neuropathy
ileus

68
Q

Vinblastine side effect

A

myelosupression

69
Q

Disproprotionately low MCV for the HB?

A

Think Beta thalassemia trait

70
Q

cyclophosphamide method of action

A

alkylating agent - cross linking DNA

71
Q

doxorubicin method of action

A

anthracycline - stabalisation of dna topoisomerase II complex

72
Q

Bleomycin method of action

A

degrades preformed dna

73
Q

stabalisation of dna topoisomerase II complex does what

A

inhibits dna and rna synthesis

74
Q

Methotrexate method of action

A

Inhibits dyhyrdrofolate reductase

75
Q

Metheotrexate side effects

A

mylosuppression
mucositits
liver fibrosis
lung fibrosis

76
Q

Vin-agents method of action

A

inhibits microtubules

77
Q

BCR-ABL test for

A

CML

78
Q

CALR test for

A

ET (along with Jak2)

79
Q

CMYC is test for

A

proto-oncogene associated with many cancers including burkitts

80
Q

PLT4 is test for

A

Platelet factor 4 (PF4 complex) is the antigen found in heparin-induced thrombocytopenia

81
Q

ET treatement

A

Hydroxycarbamide
Interferon-alpha in younger patients

82
Q

Hodgkins lymphoma - most common?

A

Nodular sclerosing

83
Q

Hogkins lymphoma - worst prognosis?

A

Lymphoctye deplete

84
Q

Hodkins lymphoma best prognosis

A

Lymphocyte predominant

85
Q

deletion of the long arm of chromosome 13 (del 13q)?

A

deletion of the long arm of chromosome 13 (del 13q) is the most common abnormality, being seen in around 50% of patients with CLL. It is associated with a good prognosis

86
Q

deletions of part of the short arm of chromosome 17 (del 17p)?

A

CLL: deletions of part of the short arm of chromosome 17 (del 17p) are seen in around 5-10% of patients and are associated with a poor prognosis

87
Q

First immunoglobulin secreted in infection

A

IG-M

88
Q

Acute promyelocytic leukaemia gene translocation?

A

t(15;17)

89
Q

t(15;17)?

A

Acute promyelocytic leukaemia

90
Q

t(12;21)

A

acute lymphoblastic leukaemia (ALL). The typical presentation of ALL is fever, hepatosplenomegaly, bone pain and bleeding

91
Q

Gene translocation in ALL

A

t(12;21)

92
Q

t(8;14)

A

NHL

93
Q

Gene translocation in NHL

A

t(8;14)

94
Q

Gene translocation in burkitts

A

t(8;22)

95
Q

t(8;22)

A

burkitts

96
Q

t(9;22)

A

CML

97
Q

inherited thombocytopenias

A

Fanconi (progressive bone marrow failure)

Wiskott-Aldrich - X linked - excema and small platlets

98
Q

Platelet type VWB

A

Abnormal Gp1b causes platelet microaggregates

99
Q

Type 2b VWD

A

Abnormal VWF causes platelet microaggregates

100
Q

What is ITP

A

Acceletrated platelet destruction due to autoantibodies to plateltts

101
Q

what is NAIT

A

Neonatal alloimmune thrombocytopenia - transplacental passage of maternal antibodies to platlets

102
Q

pregnancy associated thrombocytopenia

A

Incidental/secondary to hypertension/fatty liver - but is it HELLP??

103
Q

Megakaryocytic aplasia

A

autoimmune suppression of megakaryocyte

104
Q

Cuases of impaired production of platelets

A

Megakaryocitic aplasia
b12 folate defeciency
etoh
bone marrow infiltration/mds

105
Q

inherited causes of impaired platelet production

A

fanconis
bernard soulier (abnormal GP1b)
MyH9 disorders
Congenital amegakaryocitic thombocytopenia
wiskott-aldrich
x linked thrombocytopenia with dyserthropoiesis
Montreal syndrome
TYpe 2/platlet type VWD

106
Q

What is TTP

A

autoantibodies to ADAMST13 lead to increased VWF function and formation of platlet aggregates - rare inherited form avialable too

107
Q

thrombocytopenia broadly speaking 4 causes

A

decreased production (bone marrow/deficiencies)
increased destruction (chemo/ITP)
consumption (TTP/DIC)
Pooling in the spleen