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Block 6 > Myloproliferative diseases > Flashcards

Myloproliferative diseases Flashcards

1
Q

CML is charecterized by

A
  1. Philadelphia chromosome which is a
  2. shortened chromosome 22 resultant from 2
  3. t(9,22) that consists of a mutant gene
  4. the brc-able which produces a
  5. p210 mutant tyrosine kinase that phosphorylates intracellular targets leading to proliferation of the clone
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2
Q

clonal disorders of hematopoiesis

A
  1. acquired disorders
  2. expansion of pluripotent hematopoietic stem cell
  3. abnormal production of mature blood cells
  4. predisposition to leukemia transformation
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3
Q

Chronic myeloproliferative disorders

charecterized by:

A
  • hepatosplenomegaly
  • hypermetabolism
  • clonal increase in numbers of one or more circulating mature blood cell types
  • clonal hematopoiesis without dysplasia
  • predisposition to evolution to acute leukemia
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4
Q

Hyperluekocytosis

  • symptoms
  • signs
A

SYMPTOMS: Dyspnea, Dizziness, Slurred speech, Visual Blurriness, Diplopia, Decreased Hearing, Tinnitus, Confusion

SIGNS: Retinal Hemorrhages, Papilledema,
Priapism, Neurologic Findings

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5
Q

clinical features of CML

symptoms:

signs:

A

**symptoms: **

Fatigue
Anorexia
Abdominal Discomfort
Early Satiety
Weight Loss
Diaphoresis
Arthritis
Leukostasis
Abdominal Pain
Urticaria

** SIGNS:**

Pallor
Splenomegaly
Sternal Tenderness

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6
Q

lab findings for CML

  1. hematologic
A

Neutrophilic Leukocytosis
Anemia
Thrombocytosis
Myeloid Cells at All Stages of Development
Basophilia
Eosinophilia
Hypersegmentation
Decreased leukocyte Alkaline Phosphatase
Functional Abnormalities

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7
Q

lab findings for CML

2) marrow

A

Hypercellularity
Increased Myelopoiesis
Increased Megakaryocytes
Reticulin Fibrosis
Increased Progenitors
Cytogenetics

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8
Q

Accelerated phase of CML

definition:

path:

clinical features:

A

DEFINITION:

Transformation to a More Malignant Phenotype

**PATHOGENESIS: **

Additional Chromosomal abnormalities

Disordered Growth

 Diminished Maturation

CLINICAL FEATURES:

Fever, Diaphoresis,Weight Loss, Splenomegaly, Adenopathy, Extramedullary Blast Crisis

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9
Q

Accelerated phase of CML

  • lab features
A

Anemia
Leukopenia or Leukocytosis
Basophilia
Thrombocytopenia
Blasts (typically myloid blasts)

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10
Q

CML accelerated phase therapy

A
  • Supportive Care
    • Chemotherapy
    • Interferons
    • Leukapheresis
    • Splenectomy
    • Radiotherapy
    • Bone Marrow Transplantation
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11
Q

predictors for adverse outcome after allogenic transplant for CML

A

Advanced age of recipient
Prolonged duration of CML
Advanced stage of CML
T-cell depletion
Persistence of molecular positivity after transplant
Absence of a/c GvHD

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12
Q

Mechanism of oncogenesis for CML

A

Constitutive activation of bcr-abl tyrosine kinase

-Intracellular signaling pathway activation
Altered proliferation, adhesion, survival

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13
Q

Drug for CML

A

Imatinib mesylate

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14
Q

Therapeutic milestones in management of CML

A

Hematologic Remission
Cytogenetic Remission
Molecular Remission

Management of the sub-optimal response
- Timing of transplant viz. second-line therapy

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15
Q

therapeutic managment of CML-disease monitoring

A
  • *Hematologic**
  • Weekly until stable, and thereafter every 2-4 weeks until complete cytogenetic response is achieved, then every 4-6 weeks until molecular response, and then every 6 weeks

Cytogenetic
- Every 3-6 months until CCyR, then every 12-18months
Histopathologic
Molecular
Every 3 months

histopathologic

molecular

  • every 3 months
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16
Q

Polycythemia vera

def:

info:

A

DEFINITION:

Hematopoietic Stem Cell Disorder
Sustained Erythrocytosis

Increased RBC Mass

Cellular Proliferation

PREVALENCE: 0.5-2.6 per 100,000
AGE: Peak Onset Age 50-60
**SEX: ** Male > Female
Ethnic Predisposition: Less common in Asians, more common in Ashkenazi Jews

17
Q

Polycythemia vera

clinical features

symptoms

signs

A

**SYMPTOMS: **

insidious:

Headache

Dizziness

Vertigo

Visual Disturbances

Angina

Claudication

Early Satiety

Abdominal Pain
Pruritus
Thrombosis
Hemorrhages

**SIGNS: **

Plethora
Retinal Hemorrhages
Splenomegaly
Hepatomegaly

18
Q

Polycythemia vera

Other lab findings

A

Normal Arterial O2 Saturation (Normal FiO2)

Elevated B12 and B12- Binding Capacity

Hyperuricemia

Decreased Erythropoietin Level

Evidence of Clonality

  • G6PD isoenzyme analysis
  • JAK2 mutation
19
Q

Established criteria for P.vera

A

Elevated RCM
Absence of secondary causes
Splenomegaly-palp.
Clonality
Thrombocytosis
Neutrophilia
Splenomegaly-other
Low Epo/EEC growth

20
Q

Treatment for P. vera

A

Phlebotomy-

Goals are PCV (packed cell volume) of 45 in men, 42 in women, and 37 in late pregnancy

Radioactive Phosphorus

Other Myelosuppressive Agents

Antihistamines

Allopurinol

Aspirin, 100 mg daily is routine

21
Q

Other therapies for P. vera

A

Hydroxyurea
- Leukemogenic potential, cytopenias, mucositis

Alpha-Interferon
- Cost, inconvenience, toxicity

Anagrelide
- Mixed results on bleeding, vascular, erythromelalgia features; uncertain value viz. thrombosis. Vasodilatory effects may dissipate

  • Conventional alkylators
  • Piprobroman
22
Q

Course and prognosis for p. vera

A

Acute leukemia (1%)

“Spent Phrase” of Polycythemia Vera

Thrombosis

Special issues:
Pregnancy
Evolution to Acute Leukemia

23
Q

Primary thrombocythemia

definition

clinical features

A

DEFINITION:

  • Clonal Disorder of Platelet Production
    • Persistent Elevation in Platelet Count
    • Exclusion of Other Myeloproliferative Diseases

CLINICAL FEATURES:
Age 50-70
M=F
Usually Asymptomatic

Occasionally:

Hemorrhagic Manifestations
Erythromelalgia
Neurologic Manifestations
Thrombosis

24
Q

Lab findings in thrombocythemia

A

hematologic:

increased Hematocrit

increased RBC Mass

Normal to Increased Plasma volume

Occasionally Microcytosis

Neutrophilia

Thrombocytosis

Marrow:

Hypercellular
Increased Megakaryocytes
Myeloid and Erythroid Hyperplasia
Absent Stainable Iron

25
Q

Thrombocythemia

lab features:

A

hematologic:

Thrombocytosis > 600 x 109/Liter
Neutrophilia
Basophilia

marrow:

Hypercellularity
Increase in All Cell Lines

26
Q

Pathophysiology of essential thrombocythemia

A

Clonality is evident by G6PD isoenzyme analysis

JAK2 mutation is seen in 30-50%

EEC growth is noted

Hypersensitivity to thrombopoietic agents

Acquired MPL 515 mutation is seen in 1% of patients, often coexisting with JAK2 mutation

27
Q

Clinical course of essential thrombocythemia

A

Predictors of adverse events
- Age over 60, Leukocytosis, smoking, DM

Thrombohemorrhagic risk

  • Age over 60, platelets > 1500K, cardiovascular risk factors
  • Anagrelide/ASA associ with greater risk of arterial thrombosis, but lower risk of venous when compared to HU/ASA
  • Risk of AML transformation
28
Q

Other myeloproliferative diseases

A

Chronic idiopathic myelofibrosis

Hypereosinophilic syndrome

Chronic eosinophilic leukemia

Mastocytosis

  • Cutaneous mastocytosis
  • Systemic mastocytosis
  • Aggressive systemic mastocytosis
29
Q

Hypereosinophilic syndrome

A

Sustained eosinophilia > 1500/cu.mm

End-organ manifestations of tissue infiltration

Absence of secondary causes of eosinophila

  • Allergy
  • Metazoan parasitic infection/chronic infection
  • Hypersensitivity pneumonitis
  • Collagen vascular disease
  • Neoplasia
  • CML, Mastocytosis, AML, other MPD
30
Q

hypereosiniphilc syndrome

molecular path

A
  • Interstitial deletion of chromosome 4q12
  • FIP1L1-PDGFRα fusion tyrosine kinase
  • This fusion typically confers sensitivity to imatinib
  • Other partner genes may be PDGFRβ or c-kit, or KIF5b-PDGFRα
31
Q

Mastocytosis

A

Distinguished by site and degree of involvement

  • Cutaneous
  • Systemic
  • Aggressive systemic

Somatic clonal mutations may involve c-kit(D816V) of FIP1L1-PDGFRα

Elevated serum tryptase

Block 6 (17 decks)

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