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Kumar and Clark Haemato-oncoloy Flashcards

1
Q

What are the investigations in the Dx in acute leukaemia ?

A

FBC: To check Hb, WBC, Platelets

Blood filim: To identify morphology of cells.

Bone marrow aspiration: For immunophenotyping.

Cytogentics:FISH analysis and Molecular gentics: Prognostication.

Whole body PET or C-XR or CT CAP : To ruleout mediastinial or orther involvement.

CSF: for ALL CNS involvement.
Coagulation profile: To identify risk for DIC as in ApML.

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2
Q

Step in planning acute Leukemia therapy are ?

A

Biochemistry: Serum urate, LDH, LFT and RFT.

Cardiac function: ECG and Echo

HLA Phenotyping: for SCT

Viral testing: HIV, HBV, HCV

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3
Q

Factors of acute Leukemia supportive or active care?

A

Avoidance of symptoms of anaemia (keeping haemoglobin <80–100 g/L)

Prevention and control of bleeding

Correction of coagulation abnormalities

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4
Q

Factors affecting SCT decion making in ALL ?

A

Relapse risk and Transplant-related mortality (TRM)

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5
Q

What are the factors that determine the prognosis of AML ?

A
  • Age
  • Cytogentics
  • gene mutations
  • Presense of Minimal residual disease (MRD) after initial therapy.
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6
Q

What are the good risk or good prognosis indicators of AML?

A
  • Denovo disease
  • Favourable cytogenetics: t(15; 17) t(8; 21) or inv(16) or its variant t(16; 16)
  • CEBPA bi-allelic mutation
  • NPM1 mutation with FLT3 wild type
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7
Q

What are the poor risk or bad prognosis indicators of AML?

A

-Age >60

-Male gender

-Secondary disease, e.g. prior MDS or MPN

-High WCC

-Adverse cytogenetics: −5, del(5q), −7, abnormal 3q26 or a complex karyotype

-FLT3 internal tandem duplication mutation.

-MRD positivity post induction chemotherapy

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8
Q

APML caused by t(15,17) trasnlocation produces what gene ?

A

PML- RARA fushion gene.

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9
Q

Presentation pattern of acute Lymphoblastic Leukemia ?

A

-may present in leukaemic phase with significant marrow involvement (acute lymphoblastic leukaemia, ALL)

-May present as localized bulky disease, typically a mediastinal mass (lymphoblastic lymphoma).

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10
Q

Management of Acute Lymphoblastic Leukeima (ALL)

A
  • Remission indcution Chemotherapy
  • Remission consolidation Chemotherapy
  • Allogenic SCT in patients with high relapse risk. or Remission maintainance chemotherpay as in AML.
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11
Q

what is the Philadelphia chromosome distribution in CML?

A

Cytogentical distribution = 95%
Molecular distribution = 5%

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12
Q

CML epidemiology ?

A
  • CML accounts 14% of all leukaemias
  • It is an adult onsent male predominat disease ( age 40-60)
  • It belongs to the family of myeloproliferative neoplasms (MPNs).
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13
Q

what is the Blast crisis pattern in CML?

A
  • Myloid crisis = 75%
  • Lymphoid crisis = 25%
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14
Q

CML is often asymptomatic, when symptomatic what is the presentation?

A
  • symptomatic anaemia
  • abdominal discomfort due to splenomegaly.

-weight loss

-Fever and sweats in the absence of infection.
-headache (occasionally) or priapism due to hyperleucocytosis.

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15
Q

What are the signs of CML ?

A
  • Pallor and cynosis
    -Massive splenomegaly.
  • Lymphadenopathy suggests blast crisis.
    -extramedullary soft tissue leukemic deposit – ‘chloroma’ (indicates blast crisis).
    -retinal haemorrhage due to leukostasis
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16
Q

What are the investigations in CML ?

A

FBC: Often variable platelets, low HB.

Blood filim: significant Leukocytosis with Neutrophila, eosinophila or baseophelia.

Bone marrow aspirate: Increased cellularity is seen, with greater numbers of myeloid precursors.

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17
Q

Management of CML ?

A

-Imatinib is the firstline to indcue inhibition of enzymatic activity of BCR-ABL thyrosine kinase.

  • If resistance to imatinibe due to seconadry mutations, second-generation TKI, such as dasatinib, nilotinib or bosutinib, should be commenced.
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18
Q

How dose chronic lymphocytic Leukemia or CLL occur ?

A

It is the most common Leukemia in elderly and occurs due to clonal expansion of small B lymphocytes.

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19
Q

What is the symptomatology and epidemiology of CLL?

A
  • Most patients are asymptomatic and is diagnosed incidently.
  • Median survival is 10 years
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20
Q

What are the symptomatic forms of CLL?

A

-Leukaemic phase with significant marrow/blood involvement (CLL)
- Localized disease (small lymphocytic lymphoma, SLL)

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21
Q

CLL in all cases are prceeded by what condition?

A

Monoclonal B-cell lymphocytosis (MBL) where there are fewer than 5 × 10 ^9 /L circulating clonal B cells.

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22
Q

Rai staging system of CLL?

A

Stage 0: Low risk, Lymphocytosis alone. Therefore watch and wait.

Stage 01: Intermideiate risk, Lymphadenopathy, treat only with progression.

Stage 02: Intermediate risk- Splenomegaly- Lymphadepathy or both- treat only with progression.

Stage 03 and 4: High risk- Anemia, organomegaly and or Thrombocytopenia - treat in most cases.

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23
Q

Typical clinical features of CLL progression?

A

a )Lymphocytosis is present in all stages of the disease.

b) Progression is defined by weight loss, fatigue, fever, massive organomegaly and a rapidly increasing lymphocyte count.

c )Lymphoid areas include the cervical, axillary and inguinal lymph nodes, the spleen and the liver.

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24
Q

What are the investigations in CLL?

A
  • FBC: vairable Hb, Increased WBC, variable plateletes.
  • Blood Film: May show smudge cells, no blasts.

-Bone marrow reflects peripheral blood, often very heavily infiltrated with lymphocytes.

-Direct Coombs’ test may be positive if there is haemolysis.

  • Immunoglobulins are low or normal.
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25
What is the Lymphocytosis criteria for CLL?
WBC > 5x 10 ^9 / L
26
What is the prognosis of CLL?
The clinical course of CLL is variable. Prognosis is influenced by age and clinical stage, as well as pace of disease, measured by doubling time.
27
What is the Managment approach in CLL ?
- The major consideration is when to treat as 30% will not require any treatment. - Advanced stage disease is treated immediatly. - Intermediate stage disease has variable treatment approach.
28
What is the Supportive or active treatment in CLL
- Anaemia due to haemolysis is treated with steroids. -Anaemia and thrombocytopenia caused by marrow infiltration are treated with chemotherapy and, when necessary, transfusion. ( EPO may avid the need for transfusion). - Infection active treatment and prophylaxis should be done.
29
What is the first line treatment of CLL?
Fludarabine + Cyclophosphamide and Rithuximab.
30
what kind of Richter trasformation is seen in CLL?
- 5 to 10% of cases to DLBCL.
31
What are Myeloproliferative neoplasms?
These are stem cell disorders characterized by uncontrolled proliferation of erythroid, myeloid and/or megakaryocyte lines.
32
What are the main types of Myeloproliferative diseases ?
* polycythaemia vera (PV) * essential thrombocythaemia (ET) * myelofibrosis * chronic myeloid leukaemia (CML).
33
What is polycythaemia Vera ?
It is a clonal stem cell proliferative disorder in which there is an excessive proliferation of erythroid, myeloid and/or megakaryocytic progenitor cells.
34
What is the most common mutation in Polycythemia Vera ?
JAK2V617F in which there is point mutation causing substitution of phenylalanine for valine at position 617.
35
Clinical presentation of PV?
1) Patients are usually over 60 years ad may present with Insidious non-specific symptoms such as fatigue, itching, visual disturbance, erethromelalgia etc. 2) severe itching may occur after hot bath. 3) Gout due to increased cell turnover. 4) peptic ulcers may occur in a small subset. 5) Thrombosis and haemorrhages are major complications.
36
What are the signs of Polycythemia Vera?
1) Facial plethora 2) Splenomegaly 70% 3)Hepatomegaly 50%
37
What is the diagnostic criteria for PV ?
JAK2 -positive PV * A1: Haematocrit >0.52 in men, >0.48 in women or raised red cell mass (>25% above predicted) * A2: Mutation in JAK2.
38
What is the management of PV ?
1) Venesection: 400 to 500 ML weekly is the first step with the aim of keeping the HCT <0.45 gm / L. 2) Chemotherapy: Continuous or intermittent treatment with hydroxycarbamide (hydroxyurea). 3) JAK2 inhibitor Ruxolitinib is used to treat PV myelofibrosis,pruritus or splenomegaly. 4) Low dose Aspirin 75mg / day, if no contraindications. 5) Anagrelide: To prevent megakaryocyte differentiation and thrombocytosis.
39
PV prognosis ?
It evolves to myelofibrosis in 12 to 21% cases and AML in 5%.
40
What is Essential thrombocythaemia ?
It is a myeloproliferative neoplasm closely related to PV with significantly elevated platelets at presentation.
41
What are the gene mutations linked to ET ?
- JAK2 mutation in 50% of the cases - CALR mutation (Low risk) -MPL mutation that causes thrombopoietin receptor dysfunction ( High risk)
42
What are the causes of secondary Thrombocythemia ?
-Haematological cancers -Reactive Thrombocythemia - Hyposplenism -Drugs: Corticosteroids, Thrombopoietin receptor agonists Rebound post chemotherapy Solid malignancy
43
What is the treatment for ET?
- Aspirin for thrombosis prophylaxis. - hydroxycarbamide (hydroxyurea) for reducing platelets in high risk patients.
44
What is the therapeutic target of platelets in ET ?
400X 10^9 /L.
45
What is myelofibrosis ?
Myelofibrosis is a debilitating myeloproliferative neoplasm characterized by clonal proliferation of stem cells and abnormal myeloid cells in the bone marrow, liver, spleen and other organs. It may be primary or secondary to ET or PV.
46
What is the clinical presentation of myelofibrosis ?
* Insidious systemic symptoms of cancer. * Massive splenomegaly presented as complaint of fullness in the upper abdomen. * Perisplinits presents as complaint of severe pain during breathing.
47
What are the laboratory findings in myelofibrosis:
FBC: anaemia with leucoerythroblastic features, Leukopenia. Blood film: Poikilocytes , nucleated red cells. and teardrop cells Dry bone marrow aspiration Cytogenetic and molecular analysis: Absence of BCR-ABL mutation, Presence of JAK2 60% and CALR 25%.
48
What is the treatment for myelofibrosis ?
* Ruxolitinib to manage splenomegaly. *general supportive measures, such as blood transfusion, analgesics, antihistamines and allopurinol.
49
What is the prognosis of myelofibrosis ?
Patients may survive for 10 years or more. Poor risk factors may shorten the lifespan to 5 years.
50
What are myelodysplasias ?
They are a group of acquired bone marrow disorders caused by defects in haemopoietic stem cells. They are characterized by progressive bone marrow failure with quantitative and qualitative abnormalities in at least one of the three myeloid cell lines (red cells, granulocyte/monocytes and platelets).
51
What percentage of myelodysplasia transforms to AML
30%
52
What is the clinical presentation of myelodysplasia ?
MDS occurs mainly in the elderly and presents with symptoms of anaemia, infection or bleeding due to pancytopenia
53
When does myelodysplasia diagnosis change to AML ?
When the percentage of bone marrow blast cells increase over 20%
54
What is the treatment strategy for myelodysplasia ?
* Supportive care: red cell and platelet transfusion, bleeding and infection prevention. *EPO: for symptomatic anaemia in selected patients. * Iron chelation for secondary iron overload. *Immunosuppressive agents , such as ciclosporin and antithymocyte globulin, may be used in selected patients with a hypocellular bone marrow.
55
What is the epidemiology of Hodgkin's Lymphoma ?
~ 3/100,000/ year with a M: F ratio of 1.3: 1. - Most cases occurs b/w 16 and 65 with a peak in the third decade.
56
What is the aetiology of Hodgkins Lymphoma ?
strong association to EBV infection. 40% of cases EBV antibody.
57
What are the two major types of Hodgkins Lymphoma ?
Classical Hodgkin's Lymphoma subtypes account for 95% cases. The remaining 5% are nodular Lymphocyte predominant HL.
58
What are the sub-types of classic Hodgkin's Lymphoma ?
* Nodular sclerosing 70% * Mixed cellularity 20% * Lymphocyte rich 5% * Lymphocyte depleted rare.
59
What is the clinical presentation of Hodgkin's Lymphoma ?
* Painless cervical lymphadenopathy followed by axillary lymphadenopathy is the most common presentation. * May present with cough or SVC like presentation due to Mediastinal lymphadenopathy. * Generalised disease may present with splenomegaly and systemic B symptoms. * Pruritis and post alcohol consumption pain at the site of Lymphadenopathy.
60
What is the diagnostic work-up in HL?
* Whole body PET-CT to establish to the extent and distribution of metabolically active disease. * PET-CT also allows classification of the disease based on Cotsworld's Ann-Arbor classification. *The Hasenclever International Prognostic Score (IPS) is applied to patients with advanced-stage disease.
60
what is the first line treatment of Hodgkin's Lymphoma ?
* 2 to 4 cycles of ABVD: Doxorubicin, Bleomycin, Vinblastine and Dacarbazine + affected field radiation. *It often results in sustained remission in 90% of cases.
61
How is Nodular Lymphocyte predominant HL treated ?
It is strongly CD-20 positive and is treated differently than classical HL. It is treated with radiation alone in early stage and Rituximab for advanced stages.
62
What is the treatment of advanced HL ?
* Two cycles of ABVD followed by PET -CT to assess metabolic cure + Local radiation for bulky disease. * If there is remission Bleomycin is dropped to reduce pulmonary toxicity. * If disease is not responsive Chemo is escalated to BEACOPP: Bleomycin,Etopiside, Doxorubicn, Cyclophosphamide, Vinblastine, Procarbizine and Prednisolone.
63
What is the management of resistant and relapsing HL ?
carmustine, etoposide, cytarabine and melphalan.
64
what is the B-cell , T-cell affection pattern in NHL ?
B cells 80% and T cells 20%
65
What are the inherited conditions associated to NHL ?
* ataxia–telangiectasia and Wiskott–Aldrich syndrome.
66
What are the infections associated to NHL ?
* HIV * HTLV-1 * EBV * H. Pylori in MALT lymphoma of the GIT * Chlamydia psittaci in MALT occular lymphoma.
67
What is the pathogenesis of Non Hodgkin's Lymphoma?
Malignant clonal expansion of lymphocytes occurs at different stages of lymphocyte development, leading to the different subtypes of lymphoma.
68
What are the most common types of NHL ?
- DLBC 37% - Follicular Lymphoma 29% - MALT lymphoma 9%
69
What is the clinical presentation of follicular Lymphoma?
Painless lymphadenopathy with B symptoms. Excision biopsy for grading and screening for transformation to DLBCL( in 25% cases).
70
What is the treatment of Follicular Lymphoma ?
Since it is a B cell Lymphoma which express CD-20 chemotherapy with R-CHOP ( Rithuximab + Cyclophosphamide, Doxorubcin, Vincristine, Prednisolone.
71
What are the bad prognostic indicators of Follicular lymphoma ?
- Age > 60 - Low Hb - > 4 nodal site involvement - > LDH - Stage III or IV disease.
72
What is the clinical presentation of the most common NHL , DLBCL?
- Painless lymphadenopathy mediastinal, cervical or abdominal and B symptoms.
73
What is the treatment of DLBCL ?
- R-CHOP - If significant cardiotoxicity R-GVP in which doxorubicn is replaced by gemcitabine. - Radiotherapy after chemo for Bulky disease.
74
What is a myeloma ?
It is a neoplastic proliferation of plasma cells which present with nonfunctional paraproteins called M proteins.
75
What is Waldenström’s macroglobulinaemia?
.An IgM-producing lymphoplasmacytic lymphoma.
76
What is a paraprotein ?
It is an intact immunoglobulin, most commonly IgG (55%) or IgA (20%), and more rarely IgM or IgD.
77
What is Bence Johns protein?
It is the presence of free Light chains ( Kappa or Lambda) detected in urine of patients with Myeloma.
78
What is the serological marker of myeloma ?
Serum Free light chain.
79
What are the clinical features of Myeloma ?
- Bone pain in 60% - renal failure 30% - Hypercalcemia - Anemia - Infections - Thrombosis and Hyperviscocity - Bleeding.
80
What are the life threatening complications of Myeloma?
-Renal impairment treat with high dose dexamethasone and bisphosphonate. - Spinal cord compression : treat with radiation and dexamethasone. Hyperviscosity syndrome induced VT and AT- correct paraprotenimeia through plasmapheresis.
81
What is the bone marrow clonal plasma cells level in MGUS?
< 10%
82
What is the bone marrow plasma cell concentration in Smouldering myeloma ?
10 to 60%.
83
What is the bone marrow plasma cell concentration in active myeloma ?
> 10%
84
What are the investigations in Myeloma ?
- FBS: Blood cells can vary. ESR: often high Blood film: rouleaux formation as a consequence of the paraprotein. RFT: To assess renal function Serum Ca: usually raised. B2M and LDH: Markers of prognostication and cell turnover. Serum and urine protein electrophorosis: for Bence Jones proteine and serum free Light chains. Skeletal survey: MRI whole body or CT whole body.
85
What is the clinical feature of smouldering myeloma ?
There is plasma cell infiltration of bone marrow (>10%) but no end-organ damage or biomarkers of symptomatic myeloma.

PA MS (44 decks)

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