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Y4 cancer care > Haematology > Flashcards

Haematology Flashcards

1
Q

HL epidemiology

A

Men > women

Far less common than NHL

Bimodal age distribution - peaks around 20-25 and 80 years

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2
Q

HL risk factors

A

HIV

EBV

Autoimmune conditions - RA & sarcoidosis

Family history

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3
Q

HL clinical features

A

Lymphadenopathy - painless, firm, enlarged lymph nodes → most commonly found in the neck

‘B’ symptoms - unexplained fever, night sweats and unexplained weight loss

Mediastinal mass - incidental finding on imaging or present with SoB, cough, pain or SVCO

Pruritus

Hepatosplenomegaly

Malaise

Fatigue

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4
Q

HL ix

A

Diagnostic - excision biopsy of affected lymph nodes → Reed-Sternberg cells (’owl-like’ appearance)

Bloods - FBC, U&Es, LFTs, bone profile, LDH, uric acid, ESR, HIV, hep B & hep c, HTLV-1

Imaging - CXR, PET-CT, CT neck, C/A/P, MRI brain

Additional - LP and CSF analysis, echocardiogram, PFTs, bone marrow biopsy

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5
Q

HL staging

A

Lugano staging system - describes the anatomical distribution of disease & is of both prognostic and therapeutic importance

  • attempt to update the Ann-Arbor system
  • further modified by the presence/absence of a number of features:
    • ‘B’ symptoms
    • extranodal involvement
    • bulky disease
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6
Q

HL mx

A

Chemotherapy and radiotherapy are the mainstay of management in patients with cHL

  • ABVD regimen of treatment common - doxorubicin, bleomycin, vinblastine, dacarbazine

Relapsed disease - salvage chemotherapy, radiotherapy & autologous haematopoietic cell transplantation

Blood transfusion - must receive irradiated blood → reduce the risk of transfusion-associated graft-versus-host disease

Good prognosis — >75% of patients will be cured of condition

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7
Q

NHL epidemiology

A
  • Reed-Sternberg cells are not seen in NHL
  • more than 60 subtypes, a few notable ones are diffuse large B cell lymphoma, Burkitt lymphoma & MALT lymphoma
  • more common than Hodgkin lymphoma
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8
Q

NHL clinical features

A

Lymphadenopathy

B symptoms - fever, night sweats & weight loss

Pruritus

Splenomegaly

Hepatomegaly

Pancytopenia

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9
Q

NHL ix

A

Bloods - FBC, ESR, LFTs, uric acid,

CXR

CT scan

Bone marrow aspirate & trephine - routine in NHL

Lymph node biopsy

Immunophenotyping

Immunoglobulin tests

HIV testing

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10
Q

NHL prognosis

A

Low-grade - generally not curable, median survival of 10 years, often widely disseminated at presentation due to them being asymptomatic

High-grade - more aggressive but more likely to be cured, deadly without treatment

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11
Q

NHL mx

A

Low-grade - depends on the subtype present, MDT discussion

  • symptomless → likely no treatment will be given & will just be monitored
  • radiotherapy may be curative in localised disease
  • chlorambucil (chemotherapy)

High-grade - CHOP + rituximab chemotherapy regimen

  • monoclonal antibodies can be useful for B cell lymphomas
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12
Q

ALL

A
  • most common malignancy of childhood
  • occurs due to a proliferation of malignant lymphoid progenitor cells in the bone marrow
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13
Q

ALL aetiology

A

Combination of genetic susceptibility and environmental exposure

Infection (particularly viruses) may act as a triggering event

Cytogenetic features - TEL-AML fusion gene, philadelphia chromosome (results in BCR-ABL fusion gene)

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14
Q

ALL clinical features

A

Marrow failure

  • anaemia: fatigue, breathlessness, angina
  • neutropenia: recurrent infections
  • thrombocytopenia: petechiae, nose bleeds, bruising

Tissue infiltration - lymphadenopathy, hepatosplenomegaly, bone pain, mediastinal mass & testicular enlargement

Leucostasis - altered mental state, headache, breathlessness & visual changes

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15
Q

ALL ix

A

Bloods - FBC, LDH, uric acid, U&Es, bone profile & Mg, clotting screen, d-dimer, blood borne virus screen

Imaging - CXR (may show mediastinal mass), CT C/A/P, CT/MRI head

Bone marrow aspiration & biopsy - definitive diagnostic test → samples sent for cytogenetics & flow cytometry

Further tests - blood smear, pleural tap & LP

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16
Q

ALL mx

A

Referred a specialist haemato-oncology unit for specialist management

Key aspects: pre-phase therapy (reduce the risk of TLS), leucopheresis, supportive therapy (G-CSF may be given)

Induction chemotherapy - aim to achieve complete remission/complete molecular remission

Maintenance therapy

Stem cell transplant - allogeneic stem cell transplant may be considered & reduces the risk of relapse

Palliative care - should be considered in any patient undergoing treatment without curative intent/in those with disease symptoms that are difficult to manage

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17
Q

ALL complications

A

Tumour lysis syndrome

Neutropenic sepsis

SVCO

Chemotherapy side effects - mucositis, N&V, hair loss, cardiomyopathy, secondary malignancies

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18
Q

CLL

A
  • lymphoproliferative disorder of B lymphocytes, which results from an abnormal clonal expansion of B cells
  • incidence increases with age & more common in men
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19
Q

CLL genetic alterations

A

Tp53 mutation

11q and 13q14 deletions

Trisomy 12

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20
Q

CLL clinical features

A

Symptoms - weight loss, fevers, anorexia, night sweats, lethargy

Signs - lymphadenopathy, hepatomegaly, splenomegaly

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21
Q

CLL ix

A

Majority of patients with CLL are diagnosed incidentally on a FBC

Bloods - FBC, U&Es, bone profile, LFTs, blood film, haemolysis screen, immunoglobulins

Cytogenetics & immunophenotyping

Blood film - confirm the presence of lymphocytosis & characteristically shows smear or ‘smudge’ cells

Bone marrow assessment - done if concern for an alternative diagnosis

Imaging - CT C/A/P, USS, CXR

Lymph node biopsy

Virology

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22
Q

CLL mx

A

Watch and wait → used for patients with asymptomatic, indolent disease without poor prognostic factors

Chemotherapy

Small molecule inhibitors

Monoclonal antibodies

Allogenic stem cell transplantation - potential option in patients who fail chemo & BCR inhibitor therapy

Supportive care - vaccination, antibiotics for infections, IV immunoglobulin, PJP & herpes zoster prophylaxis

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23
Q

CLL complications

A

Richter transformation - formation of an aggressive lymphoma, 5-8 month median survival

Secondary infections

Autoimmune complications

Hyperviscosity syndrome

Secondary malignancies

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24
Q

AML

A

occurs due to the malignant transformation & proliferation of myeloid progenitor cells

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25
Q

AML risk factors

A

Myelodysplastic syndromes

Congenital disorders - Down’s syndrome, congenital neutropenia & Fanconi anaemia

Radiation exposure

Previous administration of chemotherapy

Toxins

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26
Q

AML clinical features

A

Presents with features of marrow failure/tissue infiltration

Marrow failure - anaemia, neutropenia, thrombocytopenia

Tissue infiltration - lymphadenopathy, hepatosplenomegaly, bone pain, gum hypertrophy, violaceous skin deposits, testicular enlargement

Leucostasis (large numbers of white cells entering the blood stream) - altered mental state, headache, breathlessness, visual changes

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27
Q

AML ix

A

Bloods - FBC, renal function, LFTs, clotting screen, d-dimer, bone profile & Mg, uric acid, LDH, blood borne virus screen

  • normocytic normochromic anaemia & thrombocytopenia & reduced reticulocyte counts are common

Blood smear - Auer rods is the classical finding on peripheral blood film

Bone marrow aspirate & biopsy - allows for definitive diagnosis; samples used for cytogenetics, immunophenotyping & flow cytometry

LP - organised if there is concern of CNS involvement

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28
Q

AML mx

A

Initial management - education & support, supportive care, cytoreduction (patients with leucostasis & raised WBC), CNS involvement

Chemotherapy

AML is most common indication for allogenic stem cell transplantation

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29
Q

CML

A

Slowly progressing cancer of the myeloid cell line

30
Q

CML genetics

A

Hallmark cytogenetic finding in CML is the Philadelphia chromosome

Results from a reciprocal translocation between chromosome 9 and chromosome 22

Abnormal chromosome 22 is characterised by the BCR-ABL1 fusion gene

31
Q

CML clinical features

A

20-40% are asymptomatic & picked up incidentally during investigations

B symptoms

Anaemia - fatigue, breathlessness, angina

Thrombocytopenia - petechiae, nose bleeds, bruising

Splenomegaly - early satiety, abdominal pain

Bone pain

Leucostasis - headache, breathlessness, visual changes, priapism

Signs - splenomegaly, hepatomegaly, lymphadenopathy

32
Q

CML ix

A

Bloods - FBC, U&Es, LFTs, bone profile & Mg, LDH, urate, metabolic profile, blood borne virus screen

Blood film - immature & mature myeloid cells are seen, proportion of blasts & basophils help to categorise the phase of disease

Metaphase cytogenetics - identify the abnormal chromosome 22

FISH - can be used to confirm the presence of BCR-ABL1 fusion

Bone marrow aspiration +/- trephine

33
Q

CML phases

A

Chronic phase - clinical features are non-specific

Accelerated phase - symptoms & features become more apparent, disease more difficult to treat and outcomes worse

Blast crisis - resembles an acute leukaemia with the rapid expansion of blasts

34
Q

CML mx

A

Tyrosine kinase inhibitors (imatinib) - blocks the enzyme created by the BCR-ABL1 fusion gene

Intensive chemotherapy - typically reserved for patients with blastic transformation

Allogenic stem cell transplantation

35
Q

MM

A

Malignant disorder of the plasma cells

Characterised by excess secretion of a monoclonal antibody → derived from a single clone of plasma cells that have undergone abnormal proliferation

36
Q

MM pathophysiology

A

Development of MGUS (monoclonal gammopathy of undetermined significance) - premalignant plasma cell disorder

  • thought to be an abnormal plasma cell response to antigen stimulus → leads to creation of plasma cell response to antigen stimulus

Progression from MGUS to MM - further cytogenetic abnormalities and changes to the bone marrow environment occur → promotes proliferation

  • associated with systemic problems due to plasma cell infiltration of the bone marrow & excess light chain secretion
37
Q

MM clinical features

A

Constitutional symptoms - weight loss, fatigue, loss of appetite and/or generalised weakness

Bone disease - lytic lesions, can lead to fractures

Impaired renal function - light chain nephropathy

Anaemia - seen in >90%, normal bone marrow destroyed by proliferation of malignant plasma cells, EPO deficiency due to renal disease

Hypercalcaemia - MM-induced bone demineralisation

Recurrent/persistent bacterial infection

SLIM-CRAB

38
Q

Emergency presentation of myeloma

A

Hyperviscosity syndrome - may develop with high paraprotein levels

  • symptoms: blurred vision, headaches, mucosal bleeding & dyspnoea due to heart failure
  • Requires urgent plasma exchange

Spinal cord compression

39
Q

When to suspect myeloma

A

Age > 60 and:

  • unexplained bone pain
  • fatigue
  • symptoms of hypercalcaemia
  • weight loss
  • symptoms of cord compression
  • symptoms of hyperviscosity
  • recurrent infections
40
Q

MM ix

A

Protein electrophoresis - quantitative test that separates proteins into different bands using an electric current

  • tells us whether there is an increased number of antibodies

Immunofixation - qualitative test that ‘fixes’ proteins in place by using antibodies

  • tells us which type of antibody is increased

Serum free light chains - elevated ratio between light chains is suggestive of myeloma

Urine electrophoresis - light chains within the serum may be filtered by the kidneys into the urine

41
Q

MM diagnostic criteria

A

Identification of a monoclonal antibody

Analysis of the bone marrow to look for a population of malignant clonal plasma cells

Further lab tests and imaging to assess for myeloma-related organ damage

Help differentiate between MGUS, asymptomatic myeloma & multiple myeloma

42
Q

MM mx

A

Incurable condition, treatments aim to increase periods of disease remission

MDT discussion

Induction therapy - initial treatment option, aim to induce remission; usually combination of three drugs

ASCT (autologous stem cell transplantation) - provides best option for long period of remission

Maintenance - used to maintain disease remission as long as possible; given post-induction/post transplant

Relapse or refractory disease - almost all patients will relapse, even if they respond to treatment; therapy indicated if a clinical relapse or rapid rise in paraproteins

43
Q

MM complications

A

Myeloma bone disease - bisphosphonates used for boney pain

Hypercalcaemia

Cord compression

Renal impairment

Anaemia

44
Q

Lymphadenopathy causes

A

Lymphadenopathy - condition of enlarged lymph nodes

Infective - infectious mononucleosis, HIV, eczema with secondary infection, rubella, toxoplasmosis, CMV, TB, roseola infantum

Neoplastic - leukaemia, lymphoma

Others - autoimmune conditions (SLE, RA), graft vs host disease, sarcoidosis, drugs: phenytoin, allopurinol, isoniazid

45
Q

Spleen functions

A

Immune surveillance

Proliferation and maturation of lymphocytes

Degradation of senescent & damaged erythrocytes

46
Q

Hyposplenism causes

A

Splenectomy

Sickle-cell

Coeliac disease, dermatitis herpetiformis

Graves’ disease

SLE

Amyloid

47
Q

Hyposplenism features on blood film

A

Howell-Jolly bodies

Siderocytes

48
Q

Splenomegaly causes

A

Massive splenomegaly - myelofibrosis, CML, malaria, Gaucher’s syndrome

Other causes - portal hypertension, haemolytic anaemia, infective endocarditis, thalassaemia, RA

49
Q

MDS

A

Group of malignant haematopoietic stem cell disorders

Affects elderly individuals, mean age of onset is 70 years

50
Q

MDS causes

A

Idiopathic

Secondary to exposure - toxins, genotoxic drugs, immunosuppressive agents, chemotherapy, radiation therapy

Genetic defects

51
Q

MDS complications

A

Pre-leukaemias, high risk of conversion to AML

Progresses slowly → most succumb to bleeding & infections before AML

52
Q

MDS clinical features

A

Asymptomatic in early stages

Fatigue (anaemia)

Infections (neutropenia)

Bleeding (thrombocytopenia)

53
Q

MDS ix

A

FBC - low RBCs, WBCs, platelets, normal/mildly elevated MCV, increased RDW

Low reticulocyte count, dysplastic RBCs, WBCs, normal platelets, 1-20%

Dysplastic cells, increased blasts

Chromosomal abnormalities, gene mutations

54
Q

MDS mx

A

Medications - TNF inhibitors, DNA methylation inhibitors

Allogeneic haematopoietic stem cell transplant

  • only curative option, for young individuals
  • if transplant not an option → blood product transfusions, infection control (supportive)
55
Q

PCV

A

polycythaemia = describes an increase in haematocrit, red cell count & Hb concentration

56
Q

PCV reactive causes

A

Reactive - occurs when the Hb is elevated secondary to a low plasma volume

  • can be seen in dehydration, chronic alcohol intake, excess diuretic use, pyrexia, D&V
  • ‘stress polycythaemia’ - refers to reactive polycythaemia that is found predominantly in middle aged men with stressful occupations and chronically reduced plasma volumes of uncertain cause
  • Gaisbock syndrome - more common in young men (smokers & associated with HTN) → reduces plasma volume, resulting in raised Hb
57
Q

PCV absolute causes

A

Absolute - classified into primary & secondary causes

  • primary - excess and uncontrolled erythrocytosis that is independent of EPO levels
    • associated with mutations in JAK2 gene → uncontrolled production of blood cells, especially RBCs
    • polycythaemia rubra vera = primary proliferative polycythaemia
  • secondary - excess RBC is driven by excess EPO
    • an appropriate rise in EPO → seen in conditions of chronic hypoxia
    • anabolic steroid use
    • inappropriate secretion of EPO:
      • renal neoplasma
      • CKS
      • cyanotic heart diseases
      • high-affinity haemoglobinopathies
58
Q

PCV clinical features

A

Fatigue

Headache

Visual disturbances

Pruritus

Erythromelalgia - painful burning sensation in the fingers & toes

Arterial & venous thrombosis

Plethora

Splenomegaly

Specific clinical features of PCV:

  • hyperviscosity symptoms - chest pain, myalgia, weakness, headache, blurred vision & loss of concentration
  • ruddy complexion
  • splenomegaly
59
Q

PCV ix

A

Bedside - pulse oximetry to look for possible secondary polycythaemia

FBC - raised haematocrit, raised Hb, raised red cell mass with low/low-normal plasma volume, raised leukocytes & platelet counts

  • neutrophil count & platelet count are usually normal in secondary polycythaemia !

Renal function & urate

Vitamin B12

EPO levels

JAK-2 mutation

Bone marrow biopsy - hypercellular bone marrow

Important to exclude CML - cytogenetics

60
Q

PCV mx

A

Possible underlying causes should be corrected

Venesection

PV:

Regular venesection

Aspirin 75mg daily

Cytoreductive therapy to suppress erythropoiesis in those where venesection isn’t sufficient/those at high risk of thrombosis

  • 1) hydroxycarbamide - suppresses erythrocytosis & causes a macrocytosis
  • 2) interferon, JAK2 inhibitors (first line in younger patients)
  • 3) bulsulfan

Other interventions - allopurinol (hyperuricaemia), antihistamines, SSRIs or interferon (for pruritus)

61
Q

ET

A

myeloproliferative disorder caused by dysregulated megakaryocyte (platelet precursor) proliferation

62
Q

ET epidemiology

A

Women aged 50-70 years

Median survival is 10-15 years

63
Q

ET clinical features

A

50% of patients are asymptomatic with only an incidental FBC finding

Thrombosis (arterial or venous)

Bleeding

Hyperviscosity-related → dizziness/syncope, headache

Splenomegaly

Hyposplenism

Erythromelalgia (red/blue discolouration of the extremities, often accompanied by a burning pain)

Livedo reticularis (net-like purple rash)

64
Q

ET ix

A

FBC - persistent platelet count significantly >450 x 10^9/L

Genetic testing - JAK2 mutation

Trephine biopsy - hypercellular marrow & pathological megakaryocytic clumping

65
Q

ET mx

A

Based following risk stratification

Low risk - treat with aspirin alone

Intermediate risk - treat with either hydroxycarbamide & aspirin OR aspirin alone

High risk - hydroxycarbamide & aspirin

Can also used hydroxyurea & anagrelide (specialist platelet-lowering agent)

66
Q

Myelofibrosis

A

chronic myeloproliferative neoplasm characterised by the gradual replacement of normal bone marrow tissue with fibrous tissue

67
Q

Myelofibrosis aetiology

A

Associated with genetic mutations

  • JAK2
  • CALR
  • MPL
68
Q

Myelofibrosis clinical features

A

Constitutional symptoms - weight loss, fever, night sweats

Marrow failure - anaemia, recurrent infection & abnormal bleeding/bruising

Bone pain

Haemorrhage & thrombosis (less common compared to other myeloproliferative disorders)

Splenomegaly - abdominal discomfort, early satiety

Hepatomegaly

69
Q

Myelofibrosis ix

A

Blood film - poikilocytes (tear-shaped RBCs) & a leucoerythroblastic blood film

Platelet count & WCC are often high initially, with pancytopenia occurring later

Difficult to aspirate the bone marrow → resulting in a ‘dry tap’

Trephine biopsy shows evidence of fibrosis, with hypercellular tissue, reduced fat space & increased reticulin staining

Urate and LDH usually high

JAK2 is positive in 50% of cases

70
Q

Myelofibrosis mx

A

Generally an incurable condition

Allogeneic stem cell transplantation is the one exception → patients aged <70 years with good performance status & high-risk disease

JAK-2 inhibitors (ruxolitinib) can have a good effect particularly in reducing splenomegaly & treating B symptoms

Cytotoxic agents, immunomodulatory drugs, splenic irradiation & splenectomy can be used as treatments to lessen the need for transfusion & improve symptom control

71
Q

Myelofibrosis complications

A

Transformation to acute leukaemia

Splenomegaly-related complications

Cardiovascular and thrombotic events

Y4 cancer care (10 decks)

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