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Hematologic malignancies Flashcards

1
Q

Acute lymphoid leukemia pathophys

A

Lymphoid malignancy arising in the bone marrow
All the malignant cells are blasts
Most common malignancy in children
Mutation in hematopoietic stem cell –> immortality and blast arrest, lymphoid differentiation

Most commonly pre-B cell

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2
Q

ALL diagnosis

A

Symptoms of cytopenia
Sometimes see pancytopenia, or just 1/2 cell lines low
CBC and peripheral blood smear
Bone marrow aspirate and biopsy = diagnostic
Leukemia lymphoblasts lack specific morphological features, so dependent on immunophenotyping
Cytogenics: see Philadelphia chromosome in 25% of adult cases
Flow cytometry - show lymphoid blasts

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3
Q

Acute myeloid leukemia pathophys

A

Similar to ALL, but malignant cells are myeloid blasts
More common in adults
Primary (de novo) or Secondary (hematologic malignancies - MPD of MDS, or previous chemotherapy)

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4
Q

AML Diagnosis

A 
Auer rods = pathognomonic 
Symptoms of pancytopenia
sometimes can trigger DIC
Bone marrow aspirate and biopsy with special tests, like flow cytometry
Blast count >20%
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5
Q

Chronic lymphoid leukemia pathophys

A

Malignant cells are all mature lymphoid cells
Disease of older adults
Same disease as small lymphocytic lymphoma, except CLL arises in BM and SLL arises in LN

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6
Q

CLL diagnosis

A

Lymphocyte counts very high, >30% of all nucleated cells
All cells look relatively normal
Patients often asymptomatic
Symptomatic - fatigue, weight loss, anorexia
May develop cytopenia long-term
Malignant cells can spread to LN

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7
Q

Multiple myeloma pathophys

A

Malignancy of plasma cells
Massive spread leads to destruction of bone = multiple lytic lesion
Malignant cells secrete clonal immunoglobulin

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8
Q

MM diagnosis

A

Serum protein electrophoresis - single band for a single clonal antibody being overproduced
Monoclonal gammopathy (presence of M-protein), also found in urine
Bone marrow aspirate and biopsy: significantly increased plasma cells (>30%)
Plasmacytoma
Bone pain and pathologic fractures
Hypercalcemia
Renal failure - dmg from monoclonal gammopathy and hypercalcemia
Thrombocytopenia, leukopenia
Anemia - bone marrow suppression and low EPO
Recurrent infections

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9
Q

Lymphoma pathophys

A

Hematologic malignancies arising in lymphoid tissue, usually within a LN but could also be extranodal
Can spread to another LN or to BM

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10
Q

Hodgkin lymphoma

A

tumour consists mostly of reactive inflammatory cells with only rare malignant Reed-Sternberg cell lymphocytes (large multinucleated or have bilobed nucleus with prominent eosinophilic inclusion-like nucleoli)

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11
Q

Non-Hodgkin lymphoma

A

all the cells in the tumour are malignant lymphocytes

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12
Q

Lymphoma diagnosis

A

LN biopsy: excisional>needle core>fine needle aspiration
Pathology
Ann Arbor Staging:
1: single node region OR extranodal site
2: >=2 nodes OR extranodal sites on the same side of the diaphragm
3: involvement of both sides of diaphragm, including one organ or area near LN or spleen
4: disseminated involvement of >=1 extralymphatic organs, including liver, BM, lungs

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13
Q

Myelodysplastic syndrome pathophs

A

Clonal disorder affecting hematopoietic maturation
Ineffective hematopoiesis - abnormal development and many cells apoptosis
BM failure with cytopenia
Abnormal clonal cell
Can develop to AML (worse than de novo AML)

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14
Q

Myelodysplastic syndrome diagnosis

A

Anemia +/- thrombocytopenia +/- neutropenia
BM aspirate/biopsy shows hypercellular BM with dysplastic cells (e.g. abnormal nucleus)
Peripheral blood film: RBC macrocytosis with no other cause
- WBC - decreased granulocytes, abnormal morphology
Platelets: thrombocytopenia, abnormalities of size and cytoplasm

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15
Q

Myeloproliferative disorder pathophys

A

Clonal myeloid stem cell abnormalities, leading to overproduction of one or more cell lines

4 types: chronic myelogenous leukemia
Polycythemia Vera
Essential thrombocytosis
Idiopathic myelofibrosis

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16
Q

Chronic myeloid leukemia

A

myeloid malignancy arising in BM
Mutations in a hematopoietic stem cell, which must include a bcr-abl rearrangement
Most common MPD
Cells retain the capacity to mature, so see full range of myeloid maturation
Essentially every case shares the Philadelphia chromosome (t(9;22))

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17
Q

Chronic myeloid leukemia diagnosis

A

Peripheral blood smear: leukoerythroblastic - immature RBCs and granulocytes
- presence of different mid-stage progenitor cells (vs. AML)
Marrow is hypercellular
Often clustering of megakaryocytes

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18
Q

CML presentation

A

fatigue and weight loss (anemia, hypermetabolic)
massive splenomegaly (due to extramedullary hematopoiesis) with LUQ discomfort, early satiety
CBC - high neutrophil count with many immature neutrophil forms and mature neutrophils
Commonly see eosinophilia and basophilia

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19
Q

Polycythemia vera

A

Elevated RBC mass with increased white cell and platelet production
Primary: excessive RBC production from abnormal BM, without EPO stimulation (mutation of JAK2 protein binds EPO receptor, promotes signalling)

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20
Q

Polycythemia vera diagnosis

A

R/O other causes:

  • Spurious polycythemia: dehydration
  • true polycythemia - secondary polycythemia: marrow response to hypoxia/high EPO
  • hypoxia
  • high EPO

Confirm with specialized test: epo-independent stem cell colony growth assay, JAL2 mutation testing

A criteria (need at least one, + 1 more or +2 B)

  • elevated red cell mass
  • no hypoxia
  • palpable splenomegaly
  • clonal genetic abnormality other than bcr-abl
  • endogenous erythroid colony formation in vitro

B

  • thrombocytopenia
  • leukocytosis
  • BM biopsy: panmyelosis
  • low serum EPO
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21
Q

Essential thrombocythemia

A

Overproduction of platelets withou stimulus
Very high platelet count: >600
Negative Philadelphia chromosome
Acquired JAK2 mutation
ET: megakaryocyte hyperplasia
Platelets can be functional/dysfunctional
Thrombotic complications or bleeding complications

22
Q

Idiopathic myelofibrosis

A

Megakaryocytes proliferation, make platelets and platelet-derived growth factor which promotes fibrosis
Fibrosis fills marrow space

23
Q

Idiopathic myelofibrosis diagnosis

A

Blood film: leukoerythroblastosis with tear drop RBCs, nucleated RBCs, variable polychromasia, large pltaelets and megakaryocyte fragments
BM biopsy: fibrosis, atypical megakaryocytic hyperplasia, thickening and distortion of bony trabeculae (osteosclerosis)

24
Q

Initial tests for potential hematologic malignancy

A 
"Coagulation screening panel"
Liver enzymes, serum creatinine
Peripheral blood smear
bone marrow core biopsy 
Bone marrow aspirate (assess rfee cells)
Flow cytometry for tumour cell characteristics
Karyotype/cytogenetics/FISH/PCR for specific genetic traits
CSF to assess for spread
CXR for mediastinal masses/widening
CT/US abdomen if concerns of lymphoma
25
Q

Coagulation screening panel

A

PT/INR
aPTT - mixing studies for elevated PTT
CBC-D
TT - activation of fibrinogen to fibrin

26
Q

Immediate treatment of ALL (suspicion)

A

If febrile - send pan cultures, start broad-spectrum antibiotics
Preparations to transfer patient to specialist unit

27
Q

Treatment of ALL

A

1) Induction
2) Consolidation
3) Maintenance

CNS prophylaxis with intrathecal chemotherapy/radiotherapy

28
Q

Induction phase of ALL Tx

A
  • multidrug regimen for 4-6 weeks until remission
  • BM biopsy to confirm
  • persistence of blasts: very poor prognosis
  • if disease is refractory: proceed to allogenetic stem cell transplantation
29
Q

Consolidation phase of ALL Tx

A
  • elimination of residual leukemic cells (minimal residue disease), prevent drug resistance
  • modification of initial regimen, 6 weeks
30
Q

Maintenance phase of ALL Tx

A
  • less intensive chemothrerapy

- relapses: allogeneic SCT/entry into a clinical trial

31
Q

Allogenic stem cell transplantation indication

A

First relapse in high risk patients

>=2 remissions

32
Q

CNS prophylaxis

A

Initiate during induction

Intrathecal/high-dose systemic chemotherapy

33
Q

Prognosis of ALL

A

5-year survival rates/clinical remission: 80-85% for children
CR: recovery of blood counts, bone marrow with <5% blasts, resolution of organomegaly

34
Q

Factors affecting prognosis

A

Age at diagnosis: 1-9 better
WBC: greater the leukocytosis, worse the prognosis
CNS: CNS disease - worse prognosis
Immunophenotype: precursor B- cell: best prognosis
T-cell: poor
Cytogenetics: specific translocations carry a worse prognosis

35
Q

AML prognosis and treatment

A

children - 60% 5 yr
chemo +/- transplant 6 months

Acute premyelocytic leukemia (AML with t(15;17)) can be treated w/o chemotherapy
Generally 70-80% remission (most adults <60 yo)
High risk disease requires allogenic transplant for chance of cure

36
Q

t(14;18) translocation

A

loss of apoptosis

IGH constitutive promoter fuses with BCL2 (antiapoptosis protein)

37
Q

t(8;14) transolcation

A

uncontrolled cellular proliferation

IGH constitutive promoter fuses with MYC (promotes cell proliferation)

38
Q

Oncogenic viruses (lymphoma)

A 
EBV
HIV
HCV
HTLV-1
HHV-8
39
Q

EBV lymphoma pathophys

A

Infects B-cells, incorporates own DNA into host genome

40
Q

Antigen overstimulation

A

Causes lymphocytes to proliferate
Higher chance of genetic mutation, deletion, translocation

Common causes: H. pylori, C. psittaci, B. burgdoferi
autoimmune diseases - RA, SLE, celiac disease, IBD

41
Q

Immunodeficiency

A

Loss of T-cells –> shift of lymphocyte equilibrium to the right
common causes: Iatrogenic, HIV, senile (age)

42
Q

Low-grade/indolent lymphoma

A 
slow growth
defective apoptosis
small malignant lymphocytes
tx often not required
incurable
43
Q

High-grade/aggressive lymphoma

A 
tumour grows rapidly
uncontrolled cellular proliferation
large malignant lymphocytes
treatment required at the time of diagnosis
potentially curable
44
Q

Non-Hodgkin lymphoma treatment

A

CHOP

45
Q

Hodgkin lymhoma treatment

A

ABVD

46
Q

Lymphoma treatment

A

chemo - CHOP (non-H), ABVD (H)
immuno: rituximab - used in B-cell non-H
radiation

47
Q

MDS treatment

A

supportive - RBC/platelet transfusions

High-dose chemo, allogeneic bone marrow transplant

48
Q

CML phases

A

Chronic: most patients present in this phase. With treatment can have normal lab values, and feel well

Accelerated: return of symptoms, need more drugs

Blast: transformation to acute leukemia

49
Q

CML treatment

A

imatinib - TK inhibitor, against bcr-abl (first line)
2nd gen TK inhibtors
Allogeneic transplant - younger patients

50
Q

Polycythemia vera treatment

A

phlebotomy -maintain Hct < 45%
ASA
Hydroxyurea - control platelet counts

51
Q

Essential thrombocytosis treatment

A

aspirin unless contraindicated
hydroxyurea for patients with high thrombotic risk (arrest DNA replication by inhibiting production of deoxyribonucleotides)

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