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(Cancer Care) > Haem Malignancy > Flashcards

Haem Malignancy Flashcards

1
Q

Leukaemia vs lymphoma

A

Leukaemia: mainly bone marrow is affected
Lymphoma: nodal or organ based- clonal proliferation of lymphoid cells

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2
Q

Symptoms of bone marrow failure

A

Anaemia
Thrombocytopenia
Neutropenia

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3
Q

Symptoms of disease involvement

A

Lumps
Organomegaly

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4
Q

Constitutional symptoms

A

Unintentional weigh loss
Drenching night sweats
Fevers
Pruritis

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5
Q

Myeloma or high grade lymphoma

A

Hypercalcaemia: fatigue, abdo pain, n&v, constipation, confusion, headaches, polydipsia, polyuria
Hyper viscosity

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6
Q

Investigations

A

Routine: FBC, UE, LFT, CRP, Ca, haemanitics, retics, blood film, LDH, urate, B2M
Special: Ig, SFLC, PB immunophenotyping
Imaging: CT
Invasive: tissue biopsy, BM aspirate, trephine

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7
Q

ALL

A

Proliferation of lymphoid blasts: B/Tcells
Pancytopenia, bone pain, lymphadenopathy
Multi drug chemo

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8
Q

AML

A

Pancytopenia
Varied treatment, standard of care- trials
Allogeneic stem cell transplant
Auer rods- clumps of granular matter in the cytoplasm of blasts
Usually over 65- infection, anaemia, bleeding
APL: ATRA(all trans retinoids acid) helps mature immature cells

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9
Q

CML

A

High white count+splenomegaly
Cytogenic translocation 9:22 (Philadelphia) - gives rise to BCR-ABL tyrosine kinase

Tyrosine kinase inhibitors- Imatinib
Can progress into AML

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10
Q

CLL

A

High numbers of mature B lymphocytes
Can have Nodal, splenic disease
Immune dysregulation: AIHA, ITP
Low immunoglobulins- infections
Chemo, targeted immune treatments- ibrutinib
Smudge cells- damaged lymphocytes
Richter transformation: development of aggressive high grade B cell lymphoma

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11
Q

AIHA

A

Autoimmune haemolytic anaemia
Antibody against RBC
Breathlessness, tiredness, palpitations, CP, headache, jaundice, dark urine, gallstones, splenomegaly
Steroids, rituximab,

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12
Q

ITP

A

Immune thrombocytopenic purpura
Shortage of platelets- bruising
Steroids

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13
Q

Myeloproliferative neoplasm

A

Chronic proliferation of myeloid cells
Polycythemia/thrombocytopenia/leukaemia

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14
Q

Essential thrombocythaemia

A

Thrombosis both arterial and venous
Haemorrhage
Splenomegaly
Can transform to myelofibrosis or AML
Cytoreduction in high risk patients

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15
Q

Polycythaemia rubra Vera

A

• Uncontrolled production of red cells in bone marrow despite erythropoietin
production being switched off
• Most commonly caused by JAK2 mutation • Thrombosis
• Viscosity symptoms- Headaches/drowsiness/ transient visual disturbances
• Pruritis – especially after a warm bath
• Skin – plethoric complexion
• Leukaemic transformation
• Myelofibrosis – 10-20% of patients after 15 years

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16
Q

Myelofibrosis

A

• Clonal disorder of haemopoietic stem cells
• Abnormal cells produce cytokines which cause bone marrow fibrosis
• Pancytopenia and splenomegaly
• Cytogenetic abnormalities- JAK 2 mutation
• Can transform to leukaemia
• Treatment: Supportive care, Cytoreductive drugs, JAK 2 inhibitors, Splenectomy, Bone marrow transplant

17
Q

Myelodysplasia

A

Diagnosis of exclusion
Multiple cytopenias
Clones of dysplastic cells, AML spectrum
Bone marrow biopsy is needed

18
Q

Lymphoma types

A

Hodgkin lymphoma B cell, reed sternberg cellls, only spreads contiguous manner, rare extranodal sites
Non-Hodgkin lymphoma - high or low grade, T or B cell, can spread non-contiguously, involve extranodal sites

19
Q

Lymphoma classification

A

Pathological-
follicular/
mantle cell, t(11,14)
diffuse large B cell

20
Q

Lymphoma classification
Immunepneotypic

A

• Characterising proteins or molecules
expressed by cells.
• On tissue = immunohistochemistry
• On cell suspensions = flow cytometry.
• CD antigens
CD20, CD79a (B cell)
CD3, CD4 v CD8 (T cell)
• Disease associated proteins:
Cyclin D1 (mantle cell lymphoma)
ALK (ALK+ ALCL)

21
Q

Lymphoma classification
cytogenic

A

• Specific translocations define
lymphoma subtypes.
t(11;14) – overexpression of cyclin D1 in
MCL
t(14;18) – prevents normal switching off
of BCL-2 in FL.
• Karyotyping.
• FISH.

22
Q

Lymphoma classification
sites

A

• Disease sites
Gastric MALT lymphoma
Splenic marginal zone lymphoma Cutaneous lymphoma
Hepatosplenic T cell lymphoma

23
Q

Lymphoma classification
Causes

A

Enteropathy-type T cell lymphoma (~ link with coeliac)
Post-transplant lymphoproliferative disease

24
Q

Myeloma

A

Bone marrow malignancy
Clonal population of abnormal plasma cells (B lymphocytes)
Secrete immunoglobulins- non functioning
BM infiltration then anaemia or thrombocytopenia
Cause bone&renal disease
Impaired immune system
Not curable
Rouleaux formation- aggregation of red blood cells
VCD(velcade, cyclophosphamide, dexamethosone)

25
Q

Myeloma presentation

A

Bones: generalised osteopenia, pathological fractures
Hypercalcaemia: polyuria, polydipsia, abdo pain, constipation, lethargy, confusion
BM infiltration: anaemia, thrombocytopenia, neutropenia
Infections
Hyper-viscosity: visual disturbance, headache
Neuropathy
Bleeding disorders
Associations: amyloidosis, cryoglobulinaemia, neuropathy

26
Q

Myeloma criteria

A

> 10%clonal population of BM plasma cells
Defining events:
S- 60% BM plasmacytosis
Li- light chain ratio >100
M- >1focal lesion on MRI >5mm
End organ damage
C- Ca elevation
R- renal imp
A-anaemia
B- bone lesion

27
Q

Myeloma tests

A

Immunoglobulin screen: levels of IgM, IgG, IgA- poly or mono clonal
Paraprotein: monoclonal protein, M protein
Plasma cell can only secrete kappa or lambda light chains- normal is 2:1 kappa:lambda
Serum free light chains SFLC- mono clonal : one is abnormally high compared to other, poly: both abnormally high

28
Q

Smouldering/asymptomatic myeloma

A

Paraprotein >30g/L
Clonal BM plasma cells 10-60%
Absence of any myeloma defining events or amyloidosis

29
Q

MGUS monoclonal gammopathy of undetermined significance

A

Serum Paraprotein <30g/L
Clonal BM plasma cells <10%
Absence or CRAB or amyloidosis

30
Q

Plasmacytomas

A

• Biopsy-proven lesion of bone or soft tissue with evidence of clonal
plasma cells
• Can be solitary (or have marrow involvement)
• Normal bone marrow with no evidence of clonal plasma cells (evidence of clonal plasma cells if marrow involved <10%)
• Normal imaging (except for the primary solitary lesion)
• Absence of end-organ damage (CRAB) or amyloidosis
• Plasmacytoma with marrow involvement

31
Q

Amyloidosis

A

extracellular tissue deposition of
fibrils (beta-pleated sheet)
• Immunoglobulin light chain (AL)
amyloidosis in which the fibrils are
composed of fragments of monoclonal light chains
• Patients can amyloidosis alone or in association with other plasma cell dyscrasias (multiple myeloma,
Waldenström macroglobulinemia).

• Can affect renal, liver, heart, gastrointestinal tract, or peripheral nerve involvement)
• Positive amyloid staining by Congo red in any tissue (e.g., fat aspirate, bone marrow, or organ biopsy) green
• Evidence of a monoclonal plasma cell proliferative disorder (serum monoclonal protein, abnormal free light chain ratio, or clonal plasma cells in the bone marrow)

32
Q

Waldenström’s macroglobulinaemia

A

type of non-Hodgkin’s lymphoma.
Lymphoplasmacytic lymphoma- B cells

Large amounts of a protein called immunoglobulin M (IgM)

33
Q

Lymphoma treatment

A

R-CHOP regimen for high grade lymphoma

(Cancer Care) (5 decks)

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