64 terms

Clinical Myeloproliferative Disorders w/ Malouf

STUDY
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True or False? Myeloproliferative disorders have a "multipotent hematopoietic progenitor cell" & are characterized by the proliferation of formed elements of the blood but w/out significant dysplasia
True.
Identify the 8 classifications of Myeloproliferative Diseases
True or False?

"-philia" = more (proliferating)

"-osis" = more

"-penia" = less
True
Describe some common features of myeloproliferative disorders
Describe the reasons for fatigue & malaise found w/ myeloproliferative disorders
True or False? Percussion of Traube's semilunar space is a test for Hepatomegaly
False. Splenomegaly
Identify the cells that the arrows are pointing to in the following slide
*black arrows = tear drop cells
*red arrow = myelocyte
*blue arrow = promyelocyte
*also seen: nucleated RBC, reticulocytes, normal lymphocytes, platelets (probably low in number)
True or False? CML occurs in 1.5 per 100K people yearly, occurs predominantly in women over 40 years of age
False. Slight male predominance
True or False? CML is correlated with continous low dose exposure to radiation
False. Only correlation is w/ very high-dose radiation
ID the 2 phases of CML
2 phases of CML:

*Chronic Phase

*Blast/Accelerated Phase
True or False? Fatigue & Renal Failure are the most common symptoms in CML
False. The most common findings in CML are:

*Fatigue

*Splenomegaly
Describe the less common, unique symptoms w/ CML
Describe the physical exam that will result from a PT w/ CML
True or False? Lymphadenopathy is a finding associated w/ early CML
False.
CML lab findings will include:

*Decreased WBC w/ <25% blasts

*Thrombocytosis > Thrombocytopenia

*Mild Anemia; normocytic, normochromic
False. Elevated WBC w/ <5% blasts
Describe the CML Lab findings
Describe the Treatment process for CML
True or False? In CML, allogenic stem cell transplant (SCT) is only for relief of symptoms, not curative
False. CML treatment:

*Allogenic Stem Cell Transplant (SCT) is CURATIVE.

**Many adverse effects

**1st line in acute phase
True or False? What role does Imatinib have in CML?
Describe the incidence of PV
True or False? PV is associated with exposure to high levels of radiation, such as an atomic bomb
False. There is no known Etiology -- no identifiable pattern
What is the mutation associated with PV?
PV pathophysiology involves mutation of Tyrosine Kinase JAK2
Like CMV, PV has common symptoms fatigue & splenomegaly
True.
What is the cause of Systolic Hypertension in PV?
In PV, systolic hypertension is caused by the increase in RBC w/ normal plasma volume - more fluid in the same-size vessel means higher pressure.
What is the cause of thrombosis in PTs w/ PV?
In PV, thrombosis is caused in both the arterial & venous systems due to hyperviscosity - slower flow means increased incidence of clot formation.

*most commonly the cardiac, cerebral or mesenteric vessels

*young women often present w/ intra-abdominal venous thrombosis & may occur in the hepatic vein
True or False? HTN & Thrombosis are associated w/ PV
True.
Describe Hyperviscosity syndromes associated w/ PV
True or False? Aquagenic Pruritis is associated w/ CML
False. Associated w/ PV
True or False? Unique syndromes associated w/ hyperviscosity include:
*Pruritic cutaneous lesions
*Flushing
*HA
*Dyspnea
*Syncope
False. These are some of the symptoms of Mastocytosis (not inclusive)
Describe Erythromelalgia. With what type of disorder is it associated?
PV
What leads to the Hyperuricemia associated with PV?
True or False? The physical exam w/ a PV PT will show:

*Splenomegaly
*Mild hepatomegaly
*Excoriations (scratches) - self inflicted due to puritis
*Mid-epigastric tenderness to palpation if ulcers present
True
Describe the lab findings that will return from a PT w/ PV
True or False? PV will exhibit elevated Hb & Hct
True.
True or False? PV will exhibit High EPO levels, which is part of the requirements to make a diagnosis
False. PV will have LOW EPO, & that is part of the diagnosis of PV, along w/ High Hb/Hct & JAK2 mutation
Describe the algorithm for diagnosing Polycythemia. What constitutes the test that turns suspicion to PV?
Low EPO levels is indicative of PV
True or False? Concerning PV curative treatment, the best course is allopurinol therapy
False. Allopurinol is only used to reduce uric acid levels, which is a component of PV. There are no curative therapies.
What is the most effective therapy for PV?
PV treatment:

The most effective therapy is phlebotomy to reduce the RBC count & eventually iron stores

*Goal Hgb < 14g/dL in Men

**Goal Hgb < 12g/dL in Women
Describe what therapies are available for PTs w/ PV. Is there a cure?
Where is percussion done when looking for splenomegaly?
Lowest intercostal space at the mid-axillary line
True or False? Primary Myelofibrosis si the least common of the myeloproliferative disorders
True.
True or False? Incidence of Primary Myelofibrosis is highest in adolescence
False. It's incidence increases beginning in theh 6th decade of life
Describe the pathology of Primary Myelofibrosis
True or False? Unique symptoms of Primary Myelofibrosis include:
*Night Sweats
*Mild Fever
*Weight Loss
*Joint Pain
True.
Describe the febrile process associated w/ MP disorders
Describe the joint pain associated w/ MP
True or False? Excoriations are less common w/ PM than PV or CML
True.
True or False? A bone marrow biopsy showing fibrosis is used to exclude other myeloproliferative disorders when trying to diagnose PM
True.
Describe the Lab findings associated w/ PM
True or False? A curative treatment for PM includes Allogenic Stem Cell Transplant, although it has many adverse effects
False. SCT is used for treatment of CML. There is no treatment for PM directly
What is Mastocytosis?
True or False? Mastocytosis becomes increasingly prevalent in the 6th decade of life
False. PM becomes more prevalent in the 6th decade of life. Mastocytosis mainly affects children
Describe the pathophysiology of Mastocytosis
True or False? 80% of Mastocytosis PTs progress to acute/blast phase w/in a year
False. 80% of Mastocytosis PTs have only skin involvement

*Urticaria Pigmentosa

*Mastocytoma
Describe the symptoms associated w/ Mastocytosis
Concerning the physical exam w/ a PT w/ Mastocytosis, what is a major diagnostic finding?
Darier sign: Wheal & surrounding erythema develop in a lesion after rubbing it (DIAGNOSTIC)
Describe the Mastocytosis lab findings
Describe the Treatment associated w/ Mastocytosis
Describe LCH
Langerhans Cell Histiocytosis
Describe the pathophysiology of LCH
Describe the symptoms and presentation upon physical exam associated w/ LCH
Describe the lab findings that will return from a PT w/ LCH
Describe the treatment associated w/ LCH
True or False? Myeloproliferative disorders can transform into each other & also into acute leukemia
True.