Meloproliferative Disorders Flashcards
All myeolproliferative disorders can progress to ….
May progress/transform to Acute Myeloid Leukemia (AML)
Define: Arises from the neoplastic transformation of a single hematopoietic multipotential cell
Myelofibrosis
May be Primary or Secondary
Secondary MF occurs following other myeloproliferative disorders
May evolve from Essential Thrombocytosis (or others)
2/2 atypical megakaryocytes
Pathogenesis of Myelofibrosis
Fibrous tissue develops intrameduallary
So extra medially hematopoiesis
(Spleen, liver) kick in
Leads to Hepatomegaly/ Splenomegaly
What is the smoking gun for Myelofibrosis on peripheral blood smear
Dacrocytes
Poikilocytosis
(Qualitative platelet disorder)
What is the treatment approach to Myelofibrosis
Observe and Supportive Care
-Anemia: transfusions as necessary
Immunosuppressants
-splenomegaly: 1st line hydroxyurea
Is no response: splenectomy
(Advanced dz)
What is Ruxolitinib
an oral JAK2 inhibitor, is approved by the FDA for use in patients with intermediate- or high-risk myelofibrosis
Who do we send myelofibrosis pts to
Hematology
chart on slide 18 from lecture 2, myeloproliferative disease, draw on white board
Define: -Acquired myeloproliferative disorder -overproduction of all three hematopoietic cell lines -most prominently red blood cells Results in hyperviscosity of the blood
Polycythemia Vera
-Mutation in exon 12 or 14 of JAK2 !!(V617F)
A pt presents with generalized pruritus following a warm shower or bath
Think
Polycythemia Vera
related to histamine release from basophilia
Assoc s/s
- Headache, dizziness, tinnitus, blurred vision, epistaxis
- complaints related to expanded blood volume and increased blood viscosity
What is erythomelalgia
(painful burning of the hands accompanied by erythema)
assoc. with polythemia Vera
Hallmark lab finding in Polycythemia Vera
Elevated H/H at sea level
With a normal cell morphology
What is the Dx for Polycythemia Vera
Diagnosis should be confirmed with JAK2 mutation screening
Positive in 95%-98% of patients
-The absence of a mutation in either exon 14 or exon 12 should lead the clinician to question the diagnosis
What is the mainstay of treatment for polycythemia Vera
Phlebotomy!!
+ low dose aspirin
+allopurinol for hyperuricemia
+antihistamines to manage pruritus
AVOID Alkylating Agents
(Can lead to acute leukemia)
Resistant: JAK2 Inhibitor (Ruxolitinib)
Referral for Polycythemia Vera
Refer all cases of polycythemia vera to hematology
Finding an incidental high platelet count on CBC
Think
Essential Thrombocytosis
Treat with oral hydroxyurea
Erythromelalgia
Seen in Essential Thrombocytosis and Polycythemia Vera
Live do reticularis
Seen in essential Thrombocytosis
Hallmark lab finding in essential Thrombocytosis
Isolated elevated Platlets (>2 mil.)
Treatment for Essential Thrombocytosis
Hydroxyurea
- Dosage titrated to keep platelet count < 500,000 µL
- Anagrelide (when hydroxyurea not well tolerated because of anemia)
Aspirin
81mg/day orally (rule out acquired von Willebrand syndrome first)
Erythromelalgia responds rapidly to aspirin
Strict control of coexistent cardiovascular risk factors is mandatory for all patients
Refer all cases of essential Thrombocytosis to…
hematology
If you see Philadelphia chromosome 22.. .think ..
Chronic Myelogenous Leukemia!
(CML)
Detected with PCR
What are the three phases of CML
-Chronic Phase
Does not behave like a malignant disease
Normal bone marrow function is retained
80-90% of patients present in this phase
-Accelerated Phase
Progressive anemia and thrombocytopenia
Increasing % of blasts in blood and bone marrow
-Blast Crisis
≥ 20 percent blasts in peripheral blood or bone marrow
Resembles acute leukemia
A pt presents w/ fatigue, night sweats, low grade fever, and abdominal fullness (splenomegaly), cachexia, and STERNAL tenderness..
think
Chronic Myelogenous Leukemia
-Sternal tenderness
marrow overexpansion
Particularly in the lower portion of the sternum
