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haem malignancies Flashcards

1
Q

can CML become AML

A

Yes

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2
Q

can CLL become ALL

A

no

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3
Q

what are the 2 categories of chronic myeloid disorders

A

myeloproliferative neoplasms

myelodysplastic syndrome

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4
Q

is myeloma a myeloid or lymphoid malignancy?

A

Myeloma is a lymphoid (B-cell) malignancy.

tumour of plasma cells which are B cell lineage

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5
Q

cells categorised under myeloid lineage

A

Granulocytic – neutrophils, monocytes, eosinophils, basophils,
Erythroid (RBCs)
Platelets

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6
Q

describe the development of clonal disorders of haematopoiesis

A

Genetic aberration occurring in a haematopoietic stem cell or early progenitor

Clonal expansion of the abnormal cell due to a survival or proliferative advantage

Accumulation of ‘hits’ leads to transformation to leukaemia

So both MPNs (myeloproliferative neoplasms) and MDS (myelodysplastic syndromes) can evolve into AML, and there is cross-over between MPN and MDS

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7
Q

describe the philadelphia chromosome

A

Philadelphia chromosome t(9;22)
BCR-ABL fusion protein with tyrosine kinase activity
Ph’ chromosome found in the haematopoietic stem cell

causes cell proliferation

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8
Q

clinical features of CML

A

Hypermetabolic symptoms – weight loss, anorexia, fatigue, night sweats
Splenomegaly (50%)
Features of bone marrow failure

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9
Q

lab features of CML

A

Leucocytosis – of granulocytes, especially a basophilia
Hb and platelets may be normal or reduced
Diagnosed confirmed by bone marrow biopsy and cytogenetic

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10
Q

how does imatinib work?

A

TK inhibitor
blocks downstream signalling
lifelong treatment

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11
Q

define myeloproliferative neoplasm

A

A quantitative disorder (increased)

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12
Q

define myelodysplastic syndrome

A

A disorder of both quality and
quantity (reduced)

Acquired neoplastic disorder of haematopoietic stem cells, characterised by increasing bone marrow failure with quantitative and qualitative abnormalities of all three myeloid lineages

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13
Q

examples of myeloproliferative neoplasms

A

Polycythaemia vera (PV)
Essential thrombocythaemia (ET)
Chronic myeloid leukaemia (CML)
Myelofibrosis (MF)

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14
Q

common features of myeloproliferative neoplasms

A

Clonal proliferation

Risk of progression to AML

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15
Q

define polycythaemia vera

A

Increase in red cell volume

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16
Q

symptoms of polycythaemia vera

A

Headache, dyspnoea, blurred vision, night sweats, pruritus
Plethora, bleeding
Splenomegaly

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17
Q

lab findings for polycythaemia vera

A

Raised haematocrit and Hb
WCC and platelets may also be raised
JAK2 mutation accounts for 95% of cases

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18
Q

tx of polycythaemia vera

A

Venesection to Hct <0.45
Aspirin 75mg
Cardiovascular risk factors

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19
Q

define essential thrombocytopenia

A

Sustained increase in platelet count due to megakaryocyte proliferation
Secondary causes of thrombocytosis need to be excluded

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20
Q

clinical features of essential thrombocytopenia

A

Thrombosis or haemorrhage
Erythromelalgia ( episodes of pain, redness, and swelling in various parts of the body, particularly the hands and feet)
Splenomegaly

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21
Q

management of essential thrombocytopenia

A

Cytoreduction to control platelet count – hydroxycarbamide
Aspirin
Cardiovascular risk factors

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22
Q

define myelofibrosis

A

Progressive generalised fibrosis of the bone marrow, associated with extramedullary haematopoieis (spleen and liver)
Abnormal megakaryocytes produce platelet derived growth factor (PDGF) and platelet factor 4 which stimulate the deposition of collagen and inhibit its breakdown
One third will have preceding PV

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23
Q

clinical features of myelofibrosis

A

Anaemia symptoms
Massive splenomegaly
Constitutional / hypermetabolic symptoms

Anaemia
Initially raised WCC / platelets, but as progresses develop pancytopenia

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24
Q

mx of myelofibrosis

A

Transfusion support

JAK2 inhibitor - ruxolitinib

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25
risk of MDS
progression to AML
26
lab features of MDS
Peripheral blood Red cells: macrocytosis, poikilocytosis Neutrophils: hypogranular, pseudo Pelger Platelet anisocytosis BM Hypercellular, Dysplastic morphology in 1-3 lineages, ring sideroblasts (seen with iron stain), increased blasts
27
mx of MDS
Transfusion support Growth factors – GCSF, erythropoietin Immune modulation Chemotherapy if transformation to AML Stem cell transplant
28
define AML
Aggressive clonal neoplastic disorder of the bone marrow characterised by proliferation and arrested maturation of myeloid precursor / blast cells in the bone marrow resulting in acute bone marrow failure
29
development of leukaemia
a multistep process neoplastic cell is a haematopoietic stem cell or early myeloid cell deregulation of cell growth and differentiation (associated with mutations) proliferation of the leukaemic clone with differentiation blocked at an early stage May arise de novo or transformation from MDS / MPN / CML
30
diagnosis of leukaemia
BM biopsy flow cytometry cytogenetics NGS
31
tx of AML
1. Explain diagnosis/treatment & complications 2. Supportive care: blood products, antibiotics, antifungals, Hickman line, allopurinol, specialist unit Chemotherapy: anthracycline, cytarabine, targeted therapy 4. SCT for patients with ‘high risk disease’ (age limit due to toxicity ie GVHD, infection)
32
prognosis for AML
Approximately 50% cure rate. | Prognosis in elderly inferior to young and fit patients
33
pathophys of ALL
Accumulation of lymphoid blasts
34
clinical features of ALL
BM failure, organ infiltration, including rarely testicular mass, mediastinal mass
35
lab features of ALL
Distinguished from AML by presence of lymphoid markers, eg CD19, CD7, cCD3 rather than myeloid markers, eg CD13, CD33 on flow cytometry Cytogenetics The t(9;22) translocation (Ph+) is found in 20-30% of cases and is considered a poor risk feature. Imatinib incorporated in treatment
36
treatment of ALL
Explain diagnosis/treatment & complications 2. Supportive care: blood products, antibiotics, antifungals, Hickman line, allopurinol, specialist unit Chemotherapy: - extended course 2-3 years, given in blocks of combination chemotherapy, including intrathecal methotrexate 4. SCT for patients with ‘high risk disease’ – Ph+, high WCC, adverse cytogenetics, T-ALL (age limit due to toxicity ie GVHD, infection)
37
prognosis for ALL
Curable in over 90% of children, but about 40% 5 year survival in adults Prognosis in elderly inferior to young and fit patients. Prognosis also worse at relapse.
38
define CLL (chronic lymphocytic leukaemia)
Persistent lymphocytosis with increased mature lymphocytes
39
what is the commonest leukaemia
CLL
40
clinical features of CLL
Often incidental finding on routine FBC - asymptomatic Lymphadenopathy Hepatosplenomegaly BM failure
41
lab testing for CLL
Diagnosed morphologically and with immunophenotyping – CLL score Cytogenetics – deletion or mutation of TP53 on chr 17p, associated with worse outcome Features of haemolysis Paraprotein – usually IgM/IgG
42
tx for CLL
``` Indolent, not curable Options include: Active surveillance Oral chemo Combination chemotherapy Monoclonal antibodies BCR pathway inhibitors,eg ibrutinib, idelalisib, venetoclax Splenectomy ``` Most patients not fit for SCT
43
define lymphoma
Malignant proliferation of lymphocytes that usually accumulate in lymph nodes. Can have leukaemic phase if ‘overflows’ to blood. Can infiltrate other organs
44
aetiology of lymphoma
Viral – endemic – EBV, HTLV EBV implicated in endemic Burkitt’s, HL, PTLD Immune dysregulation – HIV, immunosuppression Familial association ``` Chromosomal translocations (often involve IgH rearrangements) Gene rearrangements / mutations Pro-proliferative (eg.c-Myc) or pro-apoptotic (eg. BCL2) ```
45
clinical features of lymphoma
``` ‘B symptoms’ - Fever, night sweats, weight loss Malaise Painless lymphadenopathy Hepatosplenomegaly Mass Mass effects – eg hydronephrosis ```
46
causes of lymphadenopathy: HILLS START
HIV Infection- EBV, CMV, toxoplasmosis, pyogenic infection Lymphoma Leukaemia Sarcoidosis Syphilis TB All disseminated malignancy Rheumatoid arthritis Toxicity e.g. phenytoin
47
staging system for lymphoma
Ann arbor I 1 lymph node group II 2+ lymph node groups on the same side of the diaphragm III Lymph nodes above and below the diaphragm IV Beyond the lymph nodes, including BM A for no B symptoms, and B if B symptoms present E: involvement of a single, extranodal site, contiguous or proximal to a known nodal site (stages I to III only; additional extranodal involvement is stage IV) S: splenic involvement X: bulky nodal disease: nodal mass >1/3 of intrathoracic diameter or 10 cm in dimension
48
prognosis for lymphoma
Depends on type of lymphoma IN GENERAL: Hodgkin – aggressive, but usually curable High grade NHL – aggressive, but often curable Low grade NHL – indolent and usually treatable, but rarely curable
49
tx for lymphoma
``` Chemotherapy Immunotherapy – monoclonal Ab, eg Rituximab Small molecule inhibitors Radiotherapy Cellular therapy – CAR-T Stem cell transplant Supportive care Palliative care ``` Surgery is NOT treatment of choice
50
characteristic cells in hodgkin lymphoma
Reed sternberg
51
tx for hodgkin lymphoma
Chemotherapy: e.g ABVD (Adriamycin, Bleomycin, Vincristine, Dacarbazine) Monoclonal antibodies – eg brentuximab (anti-CD30) Checkpoint inhibitors Radiotherapy: can be curative in early stages / adjunct to chemotherapy Stem cell transplant considered in relapse – autologous and allogeneic
52
types of non-hodgkins lymphoma
Low grade: Follicular lymphoma SLL/CLL Lymphoplasmacytoid lymphoma (aka WaldenstromMacroglobulinaemia) Marginal zone lymphoma (splenic marginal zone lymphoma and MALT) Mantle Cell Lymphoma High grade: Diffuse large B-cell lymphoma (DLBCL) Burkitt lymphoma
53
tx for low grade NHL
Low grade – active surveillance, treat if symptomatic As for high grade Can undergo high grade transformation Small molecule inhibitors – BTKi, PI3Ki, BCL2i
54
tx for high grade NHL
Chemotherapy eg. CHOP (cyclophosphamide, vincristine, adriamycin, prednisolone) Monoclonal antibodies eg. Rituximab (anti-CD20) Small molecule inhibitors Radiotherapy: adjunct, palliative Cellular therapy – CAR-T cells, Stem cell transplant: autologous, allogeneic
55
define myeloma
B-cell lymphoid malignancy characterised by monoclonal expansion and accumulation of abnormal plasma cells in bone marrow
56
describe B cell maturation
Bone marrow produces naïve B-cells Travel to lymph node and are exposed to an antigen by a APC. Undergo somatic hypermutation of Ig genes Leave lymph node as plasma or ‘memory’ cells and return to bone marrow.
57
clinical presentation of myeloma
CRAB: - hyperCalcaemia - renal impairment - anaemia - bone disease Also: Recurrent / persistent bacterial infection Hyperviscosity Cord compression Features suggestive of amyloidosis Incidental finding of persistent raised ESR Coagulopathy
58
Ix for myeloma
FBC U&E, creatinine, urate, calcium, phosphate Liver function tests Immunoglobulins Serum protein electrophoresis & immunofixation Paraprotein quantification Serum Free light chain (SFLC) assay Beta-2 microglobulin, LDH Creatinine clearance 24 hour urinary protein excretion Urinary protein electrophoresis & immunofixation Quantification of Bence-Jones protein
59
what will be seen on an electrophoresis in myeloma
A monoclonal (M) band seen on protein electrophoresis
60
how can myeloma cause bone disease
``` Lytic lesions Pathological fractures Spinal cord compression Plain XR Cross-sectional imaging – MRI, CT, PET C ```
61
complications of myeloma
``` Anaemia/bone marrow failure Myeloma bone disease -pain, pathological fractures, spinal cord compression Renal failure Hypercalcaemia Hyperviscosity syndrome Infections Amyloidosis Bleeding ```
62
how does myeloma contribute to renal failure
Immunoglobulin deposition causing acute tubular necrosis Iatrogenic: NSAIDs for bone pain, chemotherapy Pyelonephritis Hypercalcaemia Hyperuricaemia Amyloid
63
tx for myeloma
Indolent, incurable, relapsing/remitting course Asymptomatic – active surveillance Steroids Targeted therapies proteasome inhibitors (bortezomib, carfilzomib, ixazomib) immunomodulatory agents (thalidomide, lenalidomide, pomalidomide) histone deacetylase (panobinostat) monoclonal antibodies (daratumamab, elotuzomab) Chemotherapy Radiotherapy Supportive care – analgesia, EPO, transfusion, dialysis Kyphoplasty
64
moa of bisphosphonates
Inhibit osteoclast activity by preventing osteoclast differentiation

IOD (54 decks)

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