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3B Specialties > Haematology > Flashcards

Haematology Flashcards

1
Q

Symptoms of Anaemia

A 
fatigue
dyspnoea
faintness
palpitations
headache
tinnitus
anorexia
(angina with underlying coronary artery disease)
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2
Q

Signs of Anaemia

A

pallor
hyperdynamic circulation- tachycardia, flow murmurs & cardiac enlargement
(rare- retinal haemorrhages)
later - heart failure- rapid transfusion here may be fatal

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3
Q

Low MCV anaemias

  • microcytic
A

iron-deficiency
thalassaemia
sideroblastic anaemia

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4
Q

Normal MCV anaemias

-normocytic

A 
acute blood loss
anaemia of chronic disease
bone marrow failure
renal failure 
hypothyroidism 
haemolysis
pregnancy
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5
Q

High MCV anaemias
- macrocytic

Megaloblastic

A

B12/ folate deficiency

cytotoxics (hydroxycarbamide)

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6
Q

Causes of iron deficiency anaemia

A

blood loss
poor diet (babies and children, special diets or poverty)
malabsorption
hook worm

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7
Q

Signs of chronic iron deficiency anaemia

A

koilonychia
atrophic glossitis
angular cheilosis
post cricoid webs

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8
Q

Blood film: iron deficiency anaemia

A

microcytic
hypochromic
with anisocytosis and poikilocytosis
- low ferritin as well

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9
Q

Treatment of iron deficiency anaemia

A
  • oral iron (se nausea, black stools)

-

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10
Q

Anaemia of chronic disease - problems

A
  1. poor use of iron in erythropoiesis
  2. cytokine induced shortening of RBC survival
  3. Decreased production of and response to EPO
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11
Q

Causes of anaemia of chronic disease

A 
chronic infection 
vasculitis 
rheumatoid
malignancy 
renal failure
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12
Q

Tests for chronic disease anaemia

A

nomocytic anaemia

ferritin normal or raised

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13
Q

Treatment of chronic disease anaemia

A

treat underlying disease more vigorously

EPO

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14
Q

Causes of macrocytic anaemia

- non-megaloblastic

A
  • alcohol
  • reticulocytosis
  • liver disease
  • hypothyroidism
  • pregancy
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15
Q

Causes of macrocytic anaemia

- other

A

myelodysplasia
myeloma
myeloproliferative disorders
aplastic anaemia

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16
Q

Causes of folate deficiency

A
  • poor diet
  • increased demand
  • malabsorption
  • drugs, alcohol, anti-epileptics, methotrexate, trimethoprim
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17
Q

Treatment of folate deficiency

A
  • treat underlying cause

- 5mg OD PO folic acid (400 micrograms/ day in pregnancy)

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18
Q

Vit B12 deficiency anaemia

General features

A
  • symptoms of anaemia, lemon tinge (pallor + jaundice), glossitis, angular cheilosis
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19
Q

Neuropsychiatric features of Vit b12 anaemia

A

irritability
depression
psychosis
dementia

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20
Q

Neurological features of Vit B 12 anaemia

A

parasthesia, peripheral neuropathy

sub acute combined degeneration of the spinal cord

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21
Q

Sub-acute combined degeneration of the spinal cord

A

insidious onset with peripheral neuropathy ( low b12)
Combination of symmetrical dorsal column loss- sensory & LMN & symmetrical corticospinal tract loss - motor & UMN

  • joint position & vibration sense go first
  • ataxia –> stiffness & weakness

Triad: 1. extensor plantars (UMN) 2. Absent knee jerks (LMN), 3. Absent ankle jerks (LMN)

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22
Q

Pernicious anaemia

A

autoimmune atrophic gastritis

- achlorhydria & lack of gastric intrinsic factor

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23
Q

Associations with pernicious anaemia

A 
other autoimmune diseases
thyroid disease 
vitiligo
addison's disease
hypoparathyroidism
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24
Q

Tests

Pernicious anaemia

A

HB- low, raised MCV, low serum B12, low/normal reticulocytes

specific tests: Parietal cell antibodies, intrinsic factor antibodies

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25
Q

Treatment for pernicious anaemia

A

Treat cause if possible

IM B12

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26
Q

haemolytic anaemia

A

intravascular

extravascular - reticuloendothelial system (macrophages of liver, spleem and bone marrow)

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27
Q

Acquired causes of haemolytic anaemia

A
  1. Immune mediated and direct anti-globulin test (Coombs) +Ve
  2. Direct antiglobulin (Coombs) test -ve
  3. Microangiopathic haemolytic anaemia
  4. Infection (malaria)
  5. Paroxysmal nocturnal haemolytic anaemia
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28
Q

Acquired causes of haemolytic anaemia

Immune mediated and direct anti-globulin test (Coombs) +Ve

A
  • drug induced
  • auto-immune haemolytic anaemia - extravascular. Warm or cold (e.g. raynaurds)
  • paroxysmal cold haemoglobulinaemia
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29
Q

Acquired causes of haemolytic anaemia

Microangiopathic haemoltic anaemia

A

mechanical disruption of RBCs in circulation
intravascular haemolysis & schistocytes
HUS, TTP, DIC, pre-eclampsia

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30
Q

Acquired causes of haemolytic anaemia

Direct antiglobulin negative

A

autoimmune hepatitis
hep b& c
post flu and vaccination
drugs - piperacillin and rituximab

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31
Q

Acquired causes of haemolytic anaemia

Paroxysmal nocturnal haemoglobulinuria

(Marchiafava-Micheli disease)

A
  • rare acquired stem cell disorder
  • haemolysis, bone marrow failure + thrombophilia
  • visceral thrombosis (hepatic, mesenteric, renal & CNS veins) and pulmonary embolism –> poor outcome
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32
Q

Hereditary causes of haemolytic anaemia

A
  1. Enzyme defects (G6PD deficiency, pyruvate kinase deficiency)
  2. Membrane defects (Coombs -ve, need folate, splenectomy) ( hereditary spherocytosis, hereditary ellipocytosis, herediatry ovalocytosis)
  3. haemoglobinopathy (sickle cell, thalassemia)
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33
Q

Hereditary causes of haemolytic anaemia

- glucose-6-phosphate dehydrogenase deficiency

A

x-linked
- mediterranean/ Africa/ Middle/Far East

oxidative crises due to decreased glutathione production, precipitated by drugs, exposure to vicia fava ( broad beans) or illness

  • rapid anamia & jaundice
  • bite and blister cells on film
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34
Q

Sickle Cell Anaemia

A

Autosomal recessive

abnormal B globin chains –> HbS instead of HbB

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35
Q

Pathogenesis of Sickle Cell Anaemia

A

HbS polymerises when deoxygenated

RBCs deform, produces sickle cell which are fragile and haemolyse- also block small vessels –> crisis

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36
Q

Tests for Sickle Cell Anaemia

A

Haemolysis is variable (Hb 60-90)
Increased reticulocytes and bilirubin
Film - sickle cells and target cells
Hb electrophoresis

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37
Q

Signs & symptoms of Sickle Cell Anaemia

Vaso occlusive

A 
- chronic haemolysis well tolerated 
common.
Triggered by cold, dehydration, infection or hypoxia. Hands & feet affected - dactylitis, 
mesenteric ischaemia, 
stroke, seizures or cognitive defects, 
avascular necrosis (femoral head), 
low flow priaprism
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38
Q

Signs & symptoms of Sickle Cell Anaemia

  • aplastic crises
A
  • due to parvovirus 19, sudden reduction in marrow production. self limiting.
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39
Q

Signs & symptoms of Sickle Cell Anaemia

  • sequestation crisis
A

mainly affects children as spleen has not yet atrophied
- pooling of blood in spleen and liver with organomegaly, severe anaemia and shock
urgent transfusion needed

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40
Q

Signs & symptoms of Sickle Cell Anaemia

Acute chest syndrome

A

pulmonary infiltrates involving complete lung segments, causing pain, fever, tachypnoea, wheeze and cough
prodromal painful crisis in the days before
chief infiltrates- fat embolism from marrow, infection with chlamydia, mycoplasma or viruses

Rx- O2, analgesia, broad spec abx, brochodilators, blood transfusion or exchange

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41
Q

Comlications of sickle cell anaemia

A
  • splenic infarction <2 (microvascular occulsion)–> increased susceptibility to infection
  • poor growth
  • chronic renal failure
  • gall stones
  • retinal disease
  • iron overload
  • blood born infections
  • lung damage (hypoxia –> fibrosis –> pulmonary hypertension
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42
Q

Management of chronic disease

Sickle cell anaemia

A
  • hydroxycarbamide if frequent crises
  • hyposlenism = prophylactic abx and immunisation
  • febrile children risk sepsis
  • bone marrow transplant may be curative
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43
Q

Managing sickle cell crises

A
  • prompt generous analgesia - IV opiates
  • cross match blood - FBC, reticulocytes, blood cultures, MSU ± CXR
  • rehydrate with IVI and keep warm
  • O2
  • measure PCV, reticulocytes, liver and spleen size twice daily
  • Transfuse if Hb/ reticulocytes fall sharply
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44
Q

Thalasaemias

A

genetic disease of unbalanced Hb synthesis
- under (or no) production of one globin chain
- Mediterranean to the Far East
point mutations, chromosome 11

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45
Q

Beta-thalassemia minor (trait)

B/B+, heterozygous state

A

Carrier
asymptomatic
mild, well tolerated anaemia, may be exacerbated by pregnancy
may be confused with iron deficiency anaemia

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46
Q

Beta- thalassemia intermedia

A

moderate anaemia not requiring transfusion

splenomegaly

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47
Q

Beta-thalassemia major

A

abnormalities in both B-globin genes
severe anaemia and failure to thribe
extramedullary haemopoiesiss occurs to anaemia causing characteristic head shape and hepatosplenomegaly
Osteopenia
‘Hair on end’ skull x-ray
life long transfusion - Fe overload may lead to endocrine failure, liver disease and cardiac toxicity

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48
Q

Treatment of thalassemias

A 
promote fitness &amp; healthy diet 
folate supplements 
2-4 weekly transfusions to keep hb>90
Iron chelation- oral deferiprone + desferrioxmine 
Ascorbic acid --> urinary excretion of iron 
- splenectomy 
- hormone replacement 
bone marrow transplant
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49
Q

alpha thalassemias

A

4 alpha-chains deleted = in utero death (Bart’s Hydrops)
3 deletions = moderate anaemia & haemolysis (hepatosplenomegaly, leg ulcers and jaundice)
2 deletions = asymptomatic carriers, low MCV
1 deletion = clinically normal

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50
Q

Causes of bleeding disorders

A
  1. vascular defects
  2. platelet disorders
  3. coagulation disorders
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51
Q

Vascular defects causing bleeding disorders

A

Congenital - Osler-Weber-Rendu syndrome, connective tissue disease (Ehler-Danlos)

Acquired: senile purpura, infection, steroids, scurvy (perifollicular haemorrhages), Henoch-SChlonlein purpura

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52
Q

Platelet disorders causing bleeding disorders

A

Decreased marrow production (aplastic anaemia, megaloblastic anaemai, marrow infiltration, marro suppession)

Excess destruction (immune, non- immune (DIC, TTP, HUS), idiopathic thrombocytopeania - antiplatelate antibodies)

Poorly functioning platelets 0 myeloproliferative diseas, NSAIDs and raised urea

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53
Q

Coagnulation disorders

A

Congenital - haemophilia, von willebran’s disease

Acquired: anti-coagulants, liver disease, DIC, vitamin K deficiency

54
Q

Haemophilia A

A

Factor VIII deficiency

x-linked

55
Q

Presentation of Heamophilia A

A

depends on severity- early in life or after surgery/ trauma with bleeds into joints - crippling arthropathy and into muscles –> haematomas (increased pressure can lead to nerve palsies and compartment syndrome)

56
Q

Diagnosis of Haemophilia A

A

Prolonged APTT

Decreased factor VIII assay

57
Q

Management of Haemophilia A

A

Avoid NSAIDs and IM injections

  • minor bleeds- pressure, elevation and desmopressin
  • major bleeds - increase factor VIII levels (50% of normal)
  • life threatening bleeds- 100% normal factor VIII with recombinant VIII
58
Q

Christmas disease

Haemophilia B

A

Factor IX deficiency
inherited
X-linked
behaves clinically like haemophilia A

59
Q

Acquired haemophilia

A

bleeding diathesis causing big mucosal bleeds in men and women
caused by sudden appearance of auto-antibodies that interfere with factor VIII

treat with steroids

60
Q

Liver disease bleeding disorder

A

decreased synthesis of clotting factors
decreased absorption of vitamin K
abnormalities of platelet function

61
Q

Malabsorption bleeding disorders

A

less uptake of vitamin K

treat with IV vitamin K or FFP for acute haemorrhage

62
Q

Incidence of AML

A

commonest acute leukaemia of adults
increases with age
can be a long term complication of chemotherapy

also associated with myelodysplastic states, radiation and Down’s syndrome

63
Q

Morphological classification of AML

A
  1. AML with recurrent genetic abnormalities
  2. AML with mutilineage dysplasia (2ndary to pre-existing myelopdysplastic syndrome
  3. AML - therapy realated
  4. AML - other
  5. Acute leukaemias of ambiguous lineage (myeloid and lymphoid phenotype)
64
Q

Symptoms of AML

A

Marrow Failure - anaemia, infection, bleeding, DIC

Infiltration - hepatomegaly, splenomegaly, gum hypertrophy, skin involvement. CNS involvement rare at presentation

65
Q

Diagnosis of AML

A

Raised WCC (but may be normal or low)
Need bone marrow biopsy
Cytogenic analysis needed for treatment recommendations

66
Q

Complications of AML

A
  • infection (neutropenic sepsis)
  • may be viral, bacterial or fungal
  • chemotherapy causes raised urate levels from tumour lysis –> allopurinol
  • leukostasis (hyperviscosity)
67
Q

Treatment of AML

A

Supportive care
Chemotherapy - daunorubicin and cytarabine
Bone marrow transplant (allogenic or autologous mobilised peripheral blood stem cell transplant)

68
Q

Bone marrow transplant

A
  • pluripotent haematopoietic stem cells collected from the marrow
  • allogenic transplant from HLA matched donor
  • complications = graft vs host disease, opportunistic infections, relapse, infertility
69
Q

ALL

A

Malignancy of lymphoid cells - B or T lymphocyte cell lines
- arrested maturation and promoting uncontrolled proliferation of immature blast cells –> marrow failure and tissue infiltration

  • genetic susceptibility + environmental trigger- ionising radiation, Down’s
  • Commonest childhood cancer
70
Q

Classification of ALL

A
  1. Morphological
  2. Immunological - precursor B-cell ALL, T-cell ALL, B-cell ALL
  3. Cytogenic - chromosomal analysis (commonly Philadelphia chromosome)
71
Q

Signs and Symptoms of ALL

A

Marrow failure: anaemia, infection, bleeding

Infiltration: Hepato-splenomegaly, lymphadenopathy- superficial or medastinal, orchidomegaly, CNS involvement - cransial nerve palsies, meningism

72
Q

Common infections in ALL

A

Chest, mouth, perianal and skin

Bacterial scepticaemia, zoster, CMV, measles, candidiasis, pneumocystis pneumonia

73
Q

Tests for ALL

A

Blasts on blood film & bone marrow
CXR & CT to look for mediastinal and abdominal lymphadenopathy
LP to look for CNS involvement

74
Q

Treatment of ALL

A

Support- blood/platelet transfusion, IV fluids, allopurinol
Infection- low threshold for sepis
Chemotherapy
Matched related allogenic marrow transplantations

75
Q

CML

A

uncontrolled clonal proliferation of myeloid cells

myeloproliferative disorder

76
Q

CML and Philadelphia

A

present in 80% of all CML
hybrid reciprocal translocation of long arm of chromosome 9 and chromosome 22

worse prognosis without philadelphia

77
Q

Symptoms of CML

A 
chronic and insidious 
weight loss
tiredness
fever
sweats
may be features of gout (purine breakdown)
Bleeding
Abdominal discomfort
78
Q

Signs of CML

A

Splenomegaly
Hepatomegaly
Anaemia
Bruising

79
Q

Tests for CML

A

Very high WCC - whole myeloid spectrum (neutrophils, myelocytes, basophils, eosinophils)
Low or normal Hb
Platelets variable
High urate and B12

80
Q

natural history of CML

A
  • variable
  • median survival 5-6 years
    chronic phase - lasts months or years. Few symptoms
    accelerated phase - increasing symptoms, spleen size, difficulty controlling counts
  • -> blast transformation - features of acute leukaemia ± death
81
Q

Treatment of CML

A

imatinib - BCR-ABL tyrosine kinase inhibitor
(dasatinib, nilotinib, bosutinib)

stem cell transplantation
if lymphoblastic transformation –> treat as ALL

82
Q

CLL

A

Accumulation of mature B cells that have escaped programmed cell death & undergone cell-cycle arrest

commonest leukaemia
Mutations, trisomies and deletions influence risk
pneumonia may be a triggering event

83
Q

Rai stage

CLL

A

0 - lymphocytosis alone. Survival >13 yrs
I - lymphocytosis + lymphadenopathy. Survival 8yrs
II - lymphocytosis + spleno/hepatomegaly. Survival 5 yrs
III - lymphocytosis + anaemia. Survival 2 yrs
IV - lymphocytosis + platelets. Survival 1 yr

84
Q

Symptoms of CLL

A

none
- often presents as a surprise finding on routine FBC
Anaemia or infection prone
if severe - weight loss, sweats, anorexia

85
Q

Signs of CLL

A

enlarged rubbery non-tender nodes
splenomegaly
hepatomegaly

86
Q

Tests for CLL

A

Raised lymphocytes- may be marked
Autoimmune haemolysis
Marrow infiltration - low Hb, neutrophils, platelets

87
Q

Complications of CLL

A
  1. Autoimmune haemolysis
  2. Infection due to hypogammaglobulinaemia - bacterial, viral esp. h. zoster
  3. Marrow failure
88
Q

Natural history of CLL

A

status quo for years of even disease regression
usually slow node enlargement
death due to infection or transformation to aggressive lymphoma (Richter’s syndrome)

89
Q

Treatment of CLL

A
  • consider drugs if: symptomatic, immunoglobulin denes are unmutated, 17p deletions
  • radiotherapy - treats lymphadenopathy
  • supportive care - transfusion, IV human immunoglobulin
    Relapsed disease - rituximab + dexamethasone (R-dex) before stem cell transplant
90
Q

Hodgkin’s Lymphoma

A

Malignant proliferation of lymphocytes
Accumulate in lymph nodes
Divided into Hodgkin’s & Non-Hodgkin’s

Hodgkin’s –> mirror image nuclei - Reed-Sternberg cells

91
Q

Incidence of Hodgkin’s Lymphoma

A 
2 peaks of incidence 
- young adults and elderly 
men to women 2:1
- increased risk if affected sibling
- EBV
- SLE
- post transplantation 
- westernisation 
- obesity
92
Q

Symptoms of Hodgkin’s Lymphoma

A
  • enlarged, painless, non-tender, rubbery superficial nodes
  • typically cervical, also axillary or inguinal
  • constitutional upset
    - weight loss, fever, night sweats, pruritus and lethargy
  • alcohol induced node pain
  • mediastinal node involvement causes features due to mass effect - SVCO, bronchial obstruction, pleural effusion

Pel-Ebstein fever implies a cyclical fever with long periods of normal/ low temp

93
Q

Signs of Hodgkin’s Lymphoma

A

Lymph node enlargement
Cachexia
Anaemia
Spleno-hepatomegaly

94
Q

Tests for Hodgkin’s Lymphoma

A

Tissue Diagnosis - lymph node excision biopsy if possible
Bloods- FBC, blood film, ESR, LFT, LDH, urate, Ca2+, raised ESR or low Hb indicate worse prognosis, LDH raised as released in cell turnover

95
Q

Staging of Hodgkin’s Lymphoma

Ann Arbor

A

I - single lymph node lesion
II - involvement of 2 + nodal areas on the same side of the diaphragm
III - involvement of nodes on both sides of the diaphragm
IV - spread beyond lymph nodes - liver or bone marrow

96
Q

Ann Arbor

A or B

A

A- no systemic symptoms other than pruritus

B - weight loss, unexplained fever or night sweats

97
Q

Chemoradiotherapy in Hodgkin’s Lymphoma

A

Radiotherapy + short course chemo

ABVD - Adriamycin, Bleomycin, Vinblastine, Dacarbazine

98
Q

Complications of Hodgkin’s Lymphoma treatment

A

Radiotherapy –> second malignancy - solid tumours, IHD, hypothyroidism and lung fibrosis

Chemo –> myelosuppression, nausea, alopecia, infection, AML, non-Hodgkins lymphoma, infertility

99
Q

Emergency presentations of Hodgkin’s Lymphoma

A

Infection

SVC Obstruction - raised JVP, sensation of fullness in head, dyspnoea, blackouts, facial oedema

100
Q

Non-Hodgkin’s Lymphoma

A

all lymphomas without Reed-Sternberg cells
most derived from B-cell lines
Diffuse Large B-Cell Lymphoma = most common

Dont all focus on nodes e.g. MALT

101
Q

Causes of non-Hodgkin’s Lymphoma

A

Immunodeficiency - drugs, HIV, H. pylori, toxins, congenital

102
Q

Non-Hodgkin’s Lymphoma extra nodal disease

A

Skin
Oropharynx
Gut - gastric MALT (h. pylori), non - malt gastic
Small bowel lymphoas (immunoproliferatie small intestine disease), MALT or enteropathy associated intra-epithelial T-cell lymphoma
Bone, CNS & lung also possible sites

103
Q

Symptoms of Non-Hodgkin’s Lymphoma

A

fever
night sweats
weight loss (less common than in Hodgkins - indicates disseminated disease)
Pancytopenia - from marrow involvement - anaemia, infection, bleeding

104
Q

Tests for Non-Hodgkin’s Lymphoma

A

FBC, U&E, LFTs, (raised LDH = worse prognosis)
Marrow and node biopsy for classification
Ann Arbor Staging

105
Q

Mangement of Non-Hodgkin’s Lymphoma

A

Low-grade - indolent, incurable, widely disseminated

High-grade- more aggressive but often curable- rapidly enlarging lymphadenopathy with systemic symptoms

106
Q

Treatment of Non-Hodgkin’s Lymphoma

A

Low grade- depends on symptoms. May need nothing, radiotherapy may be curative

High grade - R-CHOP. Granulocyte colony stimulating factors help neutropenia.

107
Q

Pancytopenia

A

reduction in all major cell lines

  • reduced marrow production
  • increased peripheral destruction (hypersplenism)
108
Q

Agranulocytosis

A

granulocytes- wbcs with neutrophil/ basophil/ eosinophil granules have stopped being made
Neutropenia

109
Q

Aplaastic anamia

A

rare stem cell disorder –> pancytopenia and hypoplastic marrow
presents with anaemia, infection or bleeding.

110
Q

Myeloproliferative disorders

A

Polycythaemia
Polycythaemia rubra vera
Essential thromocythaemia
Myelofibrosis

111
Q

Polycythaemia

A
  • relative (reduced plasma volume, normal RBC mass)
  • -> due to dehydration, or chronic due to obesity, HTN, high alcohol or tobacco intake
  • absolute (increased RBC mass)
  • -> primary (Polycythaemia rubra vera) or secondary due to hypoxia - altitude, chronic lung disease, cyanotic congenital heart disease, heavy smoking, or inapporpriately high EPO secretion
112
Q

Polycythaemia Rubra Vera

A

Malignant proliferation of a clone derived from one pluripotent marrow stem cell- mutation in JAK 2 present in >90%
Excess proliferation of RBCs, WBCs and platelets –> hyperviscosity and thrombosis

113
Q

Signs of Polycythaemia Rubra Vera

A 
asymptomatic &amp; detected on FBC
signs due to hyperviscosity - headaches, dizziness, tinnitus, visual disturbance, itch after a hot bath &amp; erythromelagia
- facial plethora and splenomegaly 
- gout due to increased RBC turnover
- arterial or venous thrombosis
114
Q

Treatment of Polycythaemia Rubra Vera

A

Venesection in younger patients

Hydroxycarbamide (alpha interferon in women of childbearing age)

115
Q

Prognosis of Polycythaemia Rubra Vera

A

Variable
thrombosis and haemorrhage are the main complications
transition to myelofibrosis in 30% or acute leukaemia in 5%
Monitor FBC every 3 months

116
Q

Essential thrombocytopenia

A

clonal proliferation of megakaryocytes leading to persistently high platelets with abnormal function
causes bleeding, arterial/venous thromosis & microvascular occlusion

  • headache, atypical chest pain, hight headedness, erythromelagia. Exclude other causes of thrombocytosis

Low dose aspirin ± hydroxycarbamide

117
Q

Myelofibrosis

A

hyperplasia of megakaryocytes - produce platelet derived growth factors –> intesne marrow fibrosis and myeloid metaplasia (haemopoiesis in the spleen and liver)–> massive hepatosplenomegaly

118
Q

Presentation of myelofibrosis

A

hypermetabolic sumptoms: night sweats, fever, weight loss, abdominal discomfort due to splenomegaly, bone marrow failure

119
Q

Treatment and prognosis of myelofibrosis

A

marrow support
allogenic stem cell transplant
Prognosis 4-5 years

120
Q

Myeloma

A

abnormal proliferation of a single clone of plasma or lymphoplasmacytic cells –> secretion of Ig or Ig fragment causes dysfunction of many organs
monoclonal band or paraprotein on electrophoresis

121
Q

Myeloma classification

A

based on the Ig product
2/3 = IgG
1/3 = IgA

2/3 pts have Bence-Jones proteins in their urine

122
Q

Symptoms of myeloma

A
  • back pain
  • osteolytic bone lesions –> back ache, pathological fractures and vertebral collapse
  • hypercalcaemia
  • anaemia, neutropenia or thrombocytopenia from marrow infiltration
  • recurrent bacterial infection (from chemo)
  • renal impairment from light chain deposition
123
Q

Diagnosis of myeloma

A

high index of suspicion - back/new pain not improving
Do ESR, film and electrophoresis

  1. Monoclonal protein band in serum or urine electrophoresis
  2. Plasma cells raised on marrow biopsy
  3. Evidence of end organ damage from myeloma - hypercalcaemia, renal insufficiency or anaemia
  4. Bone lesions - a skeletal survey detects bone disease§
124
Q

Complications of myeloma

A

Hypercalcaemia
Spinal cord compression
Hyperviscosity
Acute kidney injury

125
Q

Treatment of myeloma

A

Supportive- bone pain- analgesia, bisphosphonate, anaemia- transfuse & EPO, rehydrate
Chemotherapy

126
Q

Paraproteinaemia

A

prescence in the circulation of immunoglobulins produced by a single clone of plasma cells

  1. Multiple myeloma
  2. Waldenstrom’s macroglobulinaemia
  3. Primary amyloidosis
  4. Monoclonal gammaoapthy of uncertain significance (MGUS )
  5. Paraproteinaemia in lymphoma or leukaemia
  6. Heavy chain disease- neoplastic cells produce free Ig heave chains - may progress to lymphoma
127
Q

Amyloidoisis

A

group of disorders characterised by extracellular deposits of protein in an abnormal fibrillar form resistant to degradation

proliferation of plasma cell clones –> amyloidogenic monoclonal immunoglobulins –> fibrillar light chain protein deposition –> organ failure –> death

128
Q

Organ involvement in amyloidosis

A

Kidneys- glomerular lesions - proteinuria and nephrotic syndrome
Heart- restrictive cardiomyopathy, arrhythmias, angina
Nerves- peripheral & autonomic neuropathy, carpal tunnel
Gut - macroglossia, malabsorption/ weight loss, perforation, haemorrhage, obstruction and hepatomegaly
Vascular - purpura esp. peri-orbital

129
Q

What is thrombophilia

A

inherited or acquired coagulopathy predisposing people to thrombosis

130
Q

Inherited causes of thrombophilia

A
  • activated protein c resistance/ Factor V Leiden
  • Prothrombin gene mutation
  • Protein C & S deficiency
  • Antithrombin deficiency
131
Q

Acquired causes of thrombophilia

A
  • 3rd generation progesterones in COCP

- antiphospholipid syndrome

3B Specialties (7 decks)

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