Myelodysplastic/Myeloproliferative disorders Flashcards Preview

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Flashcards in Myelodysplastic/Myeloproliferative disorders Deck (16)
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1
Q

Overview of Myelodysplastic syndrome

A

Lots of new cases every year, Men, Elderly, Phenotype is peripheral cytopenias with normal bone marrow, risk to transform to leukemia (AML specifically)

2
Q

What are risk factors for MDS?

A

Most are unknown cause

Secondary MDS: Genetic factors, Environmental, Antineoplastic drugs

3
Q

What are the signs and symptoms of MDS?

A

Anemia (Low RBC), Infections (Low Neutrophil), bleeding (Low Platelet), Organomegaly (spleen), Sweet’s Syndrome (red lesions on body)

4
Q

What are the lab features of MDS?

A

Bone marrow: should be performed if unexplained cytopenia, Trilineage dyspoiesis (excess blasts, abnorm WBC (bilobed neutrophil),RBC (ringed sideroblasts), GIANT platelet)
Peripheral Blood smear: iron studies and cytopenia

5
Q

What are drugs that can cause MDS?

A

Alkylating agents: longer latency, Produced MDS before progressing to AML, highly resistant to tx.

Topo Inhibitors: 1-2 years latency, progresses straight to AML and skips MDS, highly resistant to tx.

6
Q

What is the International Prognostic Scoring System IPSS?

A

It rates Marrow percentage of blasts, Karyotype, and Cytopenia to give a number to tell how long the pnt has before progression to AML or to death

7
Q

What is 5q- Syndrome?

A

It is a MDS entity
Female, 68 years old, Anemic, Low WBC, high platelets
Good prognosis, less chance to progress to AML, only MDS that can be treated with drug Lenalidomide.

8
Q

What is Chronic Myelomonocytic Leukemia (CMML)?

A

Form of MDS, increased monocytes really high in marrow, variable dysplasia,
Unique: can respond to Imatinib; othewise use azacytidine

9
Q

What is Hypocellular MDS?

A

Same clinical features as Hypercellular MDS, resembles aplastic anemia,
Tx: immunosuppressive

10
Q

What is the tx for MDS?

A

Low intensity:
- Supportive (give G-CSF response 70%, EPO)
- transfusion,
- Growth factor to stimulate Hbg
High Intensity:
- Stem cell transplant, - ideally do this in pnts under 40, but most MDL is in 70s. do in people who have lower IPSS score
- chemo, (Hypomethylating agents - azacytidine, Decitabine)
- novel tx

11
Q

Myeloproliferative Disorders

A

Myeloproliferative Neoplasms: overproduction of one or more cell lines (RBC, WBC, etc), risk for leukemia transformation

Myeloproliferative disorders: 
Chronic Myeloid Leukemia (CML), 
Polycythemia Vera
Essential Thrombocytosis
Primary myelofibrosis
12
Q

What are the Genetic defects associated with Myeloproliferative neoplasms?

A

Polycythemia Vera: JAK2
Essential Thrombocytosis: JAK2
CML: BCR-ABL

13
Q

CML

A

Symptoms: fatigue, nightsweats, wt loss, splenomegaly, anemia, platelet dysfunction, HYPERLEUKOCYTOSIS
Labs: Neutrophilia, and immature myeloid cells, basophilia, elevated LDH, Uric acid - risk of tumor lysis syndrome
PHases:
Chronic - most common, we want this, easy tx, BCR-ABL mutation, high ldh,
Accelerated phase: blasts 10%, not enough to be considered leukemia but still a lot, cytopenias unresponsive to therapy
Blast Phase: blood or marrow blasts >20%, technically acute leukemia

Dx: need BCR-ABL mutation, check Marrow to see abnormal size/shape, Confirm dx with FISH or RT-PCR

Tx: imatinib,
Nilotinib (can cause QT prolong), Disatinib (can cause myelosuppression) - second generation tki’s. If in accelerated or blast phase, do these last 2.

14
Q

Polycythemia Vera

A

Elevated RBC mass in absence of secondary conditions
Inc in WBC and platelets
Male, 60-65, JAK2 mut in 90% pnts
Most likely this if you have elevated Hbg, WBC and platelet count
Symptoms: Pruritis, erythromelalgia (red hands), thrombosis, GI complaints, headache, dizziness, vision change from clogging of capillaires.
Signs: HTN, splenomegaly, hepatomegaly, ulcers, gouty features, pulm HTN

Dx:
Major criteria: Hgb>18.5, presence of JAK2 mut
Minor criteria: Bone marrow with hypercellularity and trilineage growth, Serum EPO below reference
Can have one major and two minor, or one major and two minor.

Tx: Phlebotomy and low dose aspirin,
If high risk individual (>60, hx of emboli): additonal cytoreductive therapy (hydroxyurea)

15
Q

Essential Thrombocytosis

A

60 yr, women
Jak2 v617f in 40-50%
all elevated cell lines
Marrow shows mainly enlarged megakaryocytes elev. others are elevated, but not enough to be called PV, or other myeloprolif disorder
Platelet count Greater than 450/10^9/L
Secondary cause: any stress on body, splenectomy
Tx: hydroxyurea + ASA.
if failed hydroxyurea - anagrelide, IF-alpha

16
Q

Primary myelofibrosis

A

Worst prognosis of all these
survival less than 5 yrs
65 yr
Marrow fibrosis and extramedullary hematopoiesis
clinical features: iron overload, fatigue, splenomegaly, Hgb and WBC can be low, Platelet can be high
Tx: palliative and supportive