Malignant Hemopathies Flashcards

1
Q

Define malignant hemopathies.

A

Neoplasms of the hemoatopoietic and immune system

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2
Q

What is the difference between leukemia and lymphoma

A

same disease, different tumour mass distribution

  • leukemia: mainly in bone marrow + blood
    >20% blasts in bone marrow and > 5%blast in peripheral blood
  • lymphoma: extranodal or in lymph nodes
    < 20% blasts in bone marrow and < 5% blast in peripheral blood
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3
Q

What are the types of lymphoproliferative diseases?

A
  • Acute Lymphoproliferative diseases
    e.g. ALL
  • Chronic Lymphoproliferative diseases
    e.g. lymphomas: HL and NHL
  • Immunoproliferative disease: Plasma Cell Dyscrasia
    e.g. Multiple myeloma
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4
Q

What are the histological variants of HL?

A

Classic (95%) CD20+
- nodular sclerosis
- mixed cellularity
- classical HL T-Cell Rich
- lymphocytic depletion

Non-classical (5%) CD20 -
- nodular lymphocytic predominant (NLP HL)

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5
Q

What common cell is seen in histology of HL?

A

Reed Sternberg Cells (Owl Eyes)

  • 1-5 % of cell population
    Of B-cell origin in undetermined differentiation state
  • the rest are pleomorphic cells plasmocytes, eosinophils basophils, granulocytes, macrophages and fibroblasts

-variants: hodgkin cell, popcorn, lacunar variant

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6
Q

What is NHL?

A
  • group of extremely heterogenous chronic lymphoproliferative disease differing biologically, clinically and prognostically
  • 99% malignant
  • cell of origin of B, T, NK type
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7
Q

What is the clinical classification of NHL?

A

Indolent
- slow and indolent growth
- incurable but long survival
- with periods of remission and relapse
e.g. SLL, CLL, Follicular Lymphoma

Aggressive
- aggressive, tx necessary or lethal in 1 year
- 60% curability
e.g. diffuse large B-cell lymphoma, Mantle cell lymphoma, angioimmunoblastic T cell

Very aggressive
- highly aggressive, death in a month
e.g. burkitt lymphoma/leukemia, B-cell, T/NK-cell lymphoblastic lymphoma/leukemia

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8
Q

How do you diagnose lymphoma or leukemia?

A
  • Biopsy of complete lymph nodes
  • If extra nodal, perform needle biopsy
  • Flowcytometry of peripheral blood or bone marrow to provide info on cell origin etc.
  • Definitive diagnosis: immunohistochemistry
    PCR for genetic rearrangements
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9
Q

What is the Ann Arbor Lymphoma Staging? Include A, B, E & V+

A
  • Stage I: one area of lymph node involvement
  • Stage 2: one side of diaphragm and more than 2 lymph node groups
  • Stage 3: Both sides of diaphragm and more than 2 lymph node groups
  • Stage 4: Disseminated spread into one or more extra-lymphatic organs independent of lymph node group involvement

A- no symptoms

B- B symptoms: malaise, fatigue, night sweats, loss of weight (10kg in 6 months)

E- extranodal disease

V+- bulky disease = tumour mass longest diameter > 7cm

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10
Q

What are the lab findings for lymphoma?

A
  • CBC: leukocytopenia, anaemia and eosinophilia
  • Assess virological studies e.g. HIV, EBV, HCV
  • Immunological status: plasma Ig, electrophoresis and immunoelectrophoresis of plasma proteins
  • Serum study: hypercalcemia and increase LDH
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11
Q

According to EORTC and GHSG group, when is HL advanced stage?

A

EORTC - Stage IIIA

GHSG - Stage IIB

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12
Q

What are the treatment plans for limited HL?

A

2 cycles ABVD
20 Gy irradiation of local LN

or

2 cycles of ABVD (can also be PET-CT guided)

*if positive 2 cycles of escalated BEACOPP then 30Gy irradiation

*if negative 1 cycle of ABVD and 20Gy irradiation

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13
Q

What is ABVD?

A

1st treatment for HL

ABVD (adriamycin aka doxorubicin, bleomycin, vinblastine, dacarbazine)

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14
Q

What is escalated BEACOPP?

A

bleomycin, etoposide, doxorubicin, cyclophosphamide, and vincristine, prednisone and procarbazine

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15
Q

How do you treat intermediate HL?

A

same as limited but 4 cycles of ABVD and 30 Gy irradiation

*can also be PET-CT guided with 2 cycles of ABVD and increases in irradiation dose

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16
Q

How do you treat advanced HL?

A

First line when extra-nodal involvement: 2 cycles of BEACOPPesc

Follow up by PET-CT and tailor BEACOPPesc cycles accordingly e.g. PET + then 4 cycles PET - then 2 cycles

FU with PET-CT again
+ again = localised RT
- = observation

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17
Q

How do you treat refractory/remission HL?

A

salvage ChT
e.g. autologues Stem Cell Tx,
BV (Brentiuximab Vedotin)

If further relapse: allogenic Stem Cell Tx or Pembrolizumab immune

18
Q

What is the basic treatment recommendation in NHL?

A

Indolent: wait and watch strategy
-If B symptoms + fast progression then Rituximab plus chemo or Target Tx with maintenance Tx with Ritux

Aggressive: immediate tx R-CHOP

Very Aggressive: immediate treatment (R) HyperCVAD
*allogenic SC Tx in high risk lymphoma/leukemia

19
Q

What is Hyper-CVAD?

A

Indicated in very aggressive NHL:

  • cyclophosphamide
  • vincristine
  • doxorubicin
  • methotrexate
  • cytarabine
  • dexamethasone
20
Q

What is the method of follow ups for NHL and HL?

A
  • FDG PET/CT
  • PCR/FISH (fluorescein in-situ histology)
  • FU recommended every 3 months in first 2 years
  • FU every 6 months until 5 years and then annually
21
Q

What is plasma cell dyscaria?

A
  • monoclonal proliferation of plasma cells with secretion of monoclonal protein in blood and urine
22
Q

What is the CRAB criteria?

A

Used for Multiple Myeloma

OLD - old age ( approx. 70)
C - calcium elevated (hypercalcemia)
R - renal failure
A - anemia
B - bone lytic lesions

23
Q

What is diagnostics for MM?

A
  • biopsy of bone marrow aspiration
  • confirmation of monoclonal plasma cell infiltration > 10%
  • presence of Bence Jones protein in blood or urine
24
Q

What is the IMWG 2014 criteria?

A
  1. > 10 % plasma cells in bone marrow
  2. Presence of > 1 of CRAB elements
  3. Presence of > 1 plasma cell neoplasia biomarkers (> 60% PLC infiltration, FLC > 100n and > 1 focal bone lesion on MRI (> 5mm)

Smoldering Myeloma - 10-59% PLC and absence of CRAB elements

25
Q

What is the treatment plan according to eligibility for autologous stem cell transplantation?

A

Yes (fit and less than 70)
Induction VRd

Melphalan followed by ASCT

Lenalidomide maintenance

No (more than 70)
VRd

26
Q

Whats VRd and what is VTD?

A

VRD: Bortezomib, lenalidomide and dexamethasone

VTD: Brotizomib, thalidomide and dexamethasone

27
Q

What is the relaps/refractory treatment for MM?

A

Re-induction of agents not previously used
e.g.
Daratumumab (anti-CD38)
Carfilzomib (Protease inhibitor)
Secondary Auto SCT if possible

28
Q

What are the main reason for deaths in MM?

A
  • Immunosuppression
  • TE
  • Infection
29
Q

What is autologous stem cell transplantation?

A
  • transplant own hematopoietic stem cells after high dose chemo tx
  • conducted in chemosensitive tumours
  • conducted as a transfusion after HD chemo
30
Q

What are the indications for autologous SCT?

A
  • MM after 4-6 cycles of induction tx in case of CR1 or PR, age 70 with good PS
  • HL in second complete remission after salvage tx
  • NHL of B cell origin in second complete remission after salvage tx
  • NHL of NK/T cell origin in first complete remission
  • Ewings Sarcoma: resistance to induction Tx
  • Carcinoma Embryonal Testis/Yolk sac endodermal sinus tumour: resistance to second line treatment
31
Q

What is allogenic SCT?

A
  • hematopoietic stem cells from a donor
  • after myeloablative regimen
    total body irradiation, HD cyclophosphamide/Bulsafan

*Cyclosporin, an immunosuppression drug,with MTX and anti-thymocytic glubiline, must also be given for allogenic graft control during engraftment period

graft vs leukemia effect is good
graft vs host disease is not good
-both are mediated by CD8+ T cells of donor against recipients antigens

32
Q

What are the indications for allogenic SCT?

A
  • ALL, AML: High Risk in CR1
  • HL: CR3
  • NHL of B/T/NK cell origin: CR3
  • CML: very rare
  • Myelodysplastic syndrome: high risk CR1
33
Q

What are the types of plasma cell dyscrasia?

A

-MGUS-monoclonal gammopathy of undetermined significance

-Solitary plasmocytoma (osseal, extramedullary)

-Myeloma multiplex

-Plasma cell leukemia

34
Q

What is MGUS?

A
  • this is a pretty benign condition
  • pre cancerous
  • potential to become MM after 30 ish years
35
Q

What are the chronic lympoproliferative diseases?

A

Lymphomas (Hodgkin and non-Hodgkin B, T/NK-cell origin)

36
Q

What are acute lymphoproliferative diseases?

A

Acute lymphoblastic leukemia/ lymphoblastic lymphoma of B, T/NK-cell origin

37
Q

What are acute myeloproliferative diseases?

A

-Acute myeloid leukemia (AML)
-Myeloid sarcoma (CHLOROMA)
-extramedullary solitar myeloid neoplasm (extramedullar AML)

38
Q

What are chronic myeloproliferative disease?

A

-Chronic myeloid leukemia (CML) Ph+ and very rarely Ph-
-Osteomyelofibrosis Ph-
-Policitemia rubra vera Ph-
-Essential trombocitemia Ph-
-Myelodisplastic proliferations

C-POEM

39
Q

What is the philadelphia chromosome?

A

Ph-Phyladelphia chromosome, translocation 9;22. Neoplasms that carries Ph chromosome are delineated as Ph positive (Ph+) other are Ph negative (Ph-)

40
Q
A