haem Flashcards
(107 cards)
1
Q
Waldenstroms triad
A
B-cell lymphoma characterised by organomegaly
pancytopenia
cold autoimmune haemolytic anaemia
2
Q
Waldenstroms pathology
A
B cells producing excess IGM
3
Q
Symptoms associated with Waldenstroms
A
Hyperviscosity Symptoms
(visual disturbance, headache)
Plus: anaemia, thrombocytopaenia, raised white cell count, organomegaly with lymphadenopathy
elevated IgM
4
Q
Myeloma most common IG band
A
IG-A
5
Q
Organomegaly in myeloma?
A
No
6
Q
MGUS has no CRAB symptoms- what are CRAB symptoms
A
hyperCalcaemia
Renal failure
Anaemia
Bone pain.
7
Q
IG- what is pentameric
A
IG-M
8
Q
Philadelphia chromosome - prognosis
A
GOOD in CML
BAD in AML/ALL
9
Q
Cyclophosphamide side effect
A
Haemorrhagic cystitis, myelosuppression, transitional cell carcinoma
10
Q
Bleomycin side effects
A
Lung fibrosis
11
Q
Anthracyclines (e.g doxorubicin) side effects
A
Cardiomyopathy
12
Q
Methotrexate side effects
A
Myelosuppression, mucositis, liver fibrosis, lung fibrosis
13
Q
Fluorouracil (5-FU) side effects
A
Myelosuppression, mucositis, dermatitis
14
Q
6-mercaptopurine side effects
A
Myelosuppression
15
Q
Cytarabine side effects
A
Myelosuppression, ataxia
16
Q
Vincristine, vinblastine side effects
A
Vincristine: Peripheral neuropathy (reversible) , paralytic ileus
Vinblastine: myelosuppression
17
Q
Docetaxel side effects
A
Neutropaenia
18
Q
Irinotecan side effects
A
Myelosupression
19
Q
Cisplatin side effects
A
Ototoxicity, peripheral neuropathy, hypomagnesaemia
20
Q
Hydroxyurea (hydroxycarbamide) side effects
A
Myelosuppression
21
Q
Interferon-gamma is produced by which cells
A
primarily by natural killer cells and T helper cells
22
Q
Interferon-beta is produced by which cells
A
Fibroblasts
23
Q
Interferon-alpha is produced by which cells
A
Interferon-alpha is produced by leucocytes
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.
25
Smudge/smear cells
CLL
26
Essential thrombocytosis , treatment of choice
Hydroxyurea
27
Extravascular haemolysis
thalassaemia
28
Siderotic granules
hyposplenism
29
Auer rods
apml
30
Target cells
liver disease
sickle-cell anaemia
Fe deficiency
31
Heinz bodies
G6PD
32
anemia casued by hypothyroidism
macrocytic
33
'tear-drop' poikilocytes
myelofibrosis
34
Intravascular haemolysis on blood film
schistocytes
35
Spherocytes
AIHA
36
Basophilic stippling
Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia
37
Howell-Jolly bodies
Hyposplenism
38
Schistocytes ('helmet cells')
Intravascular haemolysis
Mechanical heart valve
Disseminated intravascular coagulation
39
Burr cells (echinocytes)
Uraemia
Pyruvate kinase deficiency
40
Acanthocytes
Abetalipoproteinemia
41
Splenomegaly causes
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)
42
platelet transfusion in surgery targets
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
43
epidemiology of hodgkins/non-hodgkins
Non-Hodgkin's lymphoma is much more common than Hodgkin's lymphoma
44
Symptoms of NHL
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
CLL complications
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
Richters transformation in CLL>?
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
Percentages of iron in the body?
total body iron = 4g
haemoglobin = 70%
ferritin and haemosiderin = 25%
myoglobin = 4%
plasma iron = 0.1%
48
iron is stored as
ferritin
49
iron is transported by
carried in plasma as Fe3+ bound to transferrin
50
DIC tests suggestive of
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
warfarin PT/APTT/Fibrinogen/Bleeding time/PLt
PT prolonged - normal everything else
52
Asprin PT/APTT/Fibrinogen/Bleeding time/PLt
Prolonged bleeding time normal everythign else
53
DIC PT/APTT/Fibrinogen/Bleeding time/PLt
Prolonged PT/APPT low fibrinogen low platlets
54
Aplastic crisis in sickle cell
Following parvovirus infection - pancytopenia
55
Acute chest syndrome in sickle cell
pain/cough/wheeze/sob
CT pulm infiltrates
56
Sequestration crisis in sickle cell
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
Ann arbor staging I
Stage I - involves a single regional lymph node
58
Ann arbor staging II
Stage II - involves two or more lymph nodes on one side of the diaphragm
59
Ann arbor staging III
Stage III of the Ann-Arbor clinical staging of lymphomas involve lymph nodes on both sides of the diaphragm
60
Ann arbor staging IV
Stage IV - distant spread involving one or more extra lymphatic organs
61
Ann Arbor A/B
A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)
62
Lymphoma treatement
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
most common inherited thrombophilia?
Activated protein C resistance (Factor V Leiden) is the most common inherited thrombophilia
64
Bleomycin side effect
Lung fibrosis
65
Doxyrubcin side effect
Cardiomyopathy
66
Cyclophosphamide side effect
haemoraggic cystitis
TCC
67
Vincristine side effect
peripheral neuropathy
ileus
68
Vinblastine side effect
myelosupression
69
Disproprotionately low MCV for the HB?
Think Beta thalassemia trait
70
cyclophosphamide method of action
alkylating agent - cross linking DNA
71
doxorubicin method of action
anthracycline - stabalisation of dna topoisomerase II complex
72
Bleomycin method of action
degrades preformed dna
73
stabalisation of dna topoisomerase II complex does what
inhibits dna and rna synthesis
74
Methotrexate method of action
Inhibits dyhyrdrofolate reductase
75
Metheotrexate side effects
mylosuppression
mucositits
liver fibrosis
lung fibrosis
76
Vin-agents method of action
inhibits microtubules
77
BCR-ABL test for
CML
78
CALR test for
ET (along with Jak2)
79
CMYC is test for
proto-oncogene associated with many cancers including burkitts
80
PLT4 is test for
Platelet factor 4 (PF4 complex) is the antigen found in heparin-induced thrombocytopenia
81
ET treatement
Hydroxycarbamide
Interferon-alpha in younger patients
82
Hodgkins lymphoma - most common?
Nodular sclerosing
83
Hogkins lymphoma - worst prognosis?
Lymphoctye deplete
84
Hodkins lymphoma best prognosis
Lymphocyte predominant
85
deletion of the long arm of chromosome 13 (del 13q)?
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
deletions of part of the short arm of chromosome 17 (del 17p)?
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
First immunoglobulin secreted in infection
IG-M
88
Acute promyelocytic leukaemia gene translocation?
t(15;17)
89
t(15;17)?
Acute promyelocytic leukaemia
90
t(12;21)
acute lymphoblastic leukaemia (ALL). The typical presentation of ALL is fever, hepatosplenomegaly, bone pain and bleeding
91
Gene translocation in ALL
t(12;21)
92
t(8;14)
NHL
93
Gene translocation in NHL
t(8;14)
94
Gene translocation in burkitts
t(8;22)
95
t(8;22)
burkitts
96
t(9;22)
CML
97
inherited thombocytopenias
Fanconi (progressive bone marrow failure)
Wiskott-Aldrich - X linked - excema and small platlets
98
Platelet type VWB
Abnormal Gp1b causes platelet microaggregates
99
Type 2b VWD
Abnormal VWF causes platelet microaggregates
100
What is ITP
Acceletrated platelet destruction due to autoantibodies to plateltts
101
what is NAIT
Neonatal alloimmune thrombocytopenia - transplacental passage of maternal antibodies to platlets
102
pregnancy associated thrombocytopenia
Incidental/secondary to hypertension/fatty liver - but is it HELLP??
103
Megakaryocytic aplasia
autoimmune suppression of megakaryocyte
104
Cuases of impaired production of platelets
Megakaryocitic aplasia
b12 folate defeciency
etoh
bone marrow infiltration/mds
105
inherited causes of impaired platelet production
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
What is TTP
autoantibodies to ADAMST13 lead to increased VWF function and formation of platlet aggregates - rare inherited form avialable too
107
thrombocytopenia broadly speaking 4 causes
decreased production (bone marrow/deficiencies)
increased destruction (chemo/ITP)
consumption (TTP/DIC)
Pooling in the spleen
