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Hematology/Immunology Part 2 > Myeoloproliferative Disorders > Flashcards

Myeoloproliferative Disorders Flashcards

1
Q

What are Myeloproliferative Neoplasms (MPNs)?

A

Diseases caused by acquired clonal abnormalities of the hematopoietic stem cell.

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2
Q

What is the common feature of Polycythemia Vera (PV), Essential Thrombocytosis (ET), and Primary Myelofibrosis (PMF)?

A

Overproduction of one or more formed elements of the blood.

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3
Q

What major complications are associated with Myeloproliferative Neoplasms?

A

Arterial and venous thrombosis, hemorrhage, progression to other diseases.

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4
Q

What is a common mutation found in PV, ET, and PMF?

A

JAK2 mutation.

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5
Q

What is the role of the JAK2 V617F mutation in MPNs?

A

It leads to deregulation of kinase activity, contributing to cell overgrowth and inhibition of apoptosis.

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6
Q

What is Polycythemia Vera (PV)?

A

A condition characterized by the overproduction and accumulation of normal red cells, granulocytes, and platelets without a definite stimulus.

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7
Q

What is the significance of the JAK2 gene in MPNs?

A

It is a major final common signaling pathway and an appropriate therapeutic target.

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8
Q

What are the clinical features of PV, ET, and PMF?

A

Older age, higher hemoglobin and leukocyte count, more thrombosis, pruritus, and fibrosis.

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9
Q

What is the incidence rate of Polycythemia Vera?

A

2 per 100,000 people.

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10
Q

What is the median age of onset for Polycythemia Vera?

A

65 years old.

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11
Q

What other mutations can drive the JAK-STAT pathway in MPNs?

A

LNK, CALR, TET2, ASXL1, DNMT3A.

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12
Q

What genetic mutation is commonly associated with Polycythemia Vera?

A

JAK2 mutation.

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13
Q

What are some causes of absolute erythrocytosis?

A

Hypoxia, carbon monoxide intoxication, high-altitude, pulmonary disease, right-to-left shunts, sleep apnea syndrome, neurologic disease, renal disease, tumors, drugs, familial conditions.

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14
Q

What is the relationship between JAK2 V617F and leukemic transformation in MPNs?

A

Leukemic transformation can occur in a JAK2 V617F-negative type cell.

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15
Q

Why are PV, ET, and PMF classified separately from other MPNs?

A

They share common mutations in JAK2 and MPL genes and have targeted therapies developed for them.

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16
Q

What are some causes of relative erythrocytosis?

A

Loss of fluid from the vascular space, chronic plasma volume contraction, hypoxia, androgen therapy, recombinant erythropoietin therapy, hypertension, tobacco use, pheochromocytoma, ethanol abuse, sleep apnea.

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17
Q

What are the clinical features of Polycythemia Vera?

A

Erythrocytosis, splenomegaly, leukocytosis, thrombocytosis.

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18
Q

What are some symptoms of Polycythemia Vera?

A

Tinnitus, headache, visual changes, mental clouding, facial plethora, hypertension, thrombosis, digital ischemia, erythromelalgia, easy bruising, generalized pruritus.

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19
Q

What are the main treatment goals for Polycythemia Vera?

A

Reduce red cell mass and prevent thrombotic events.

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20
Q

What is the risk of progression to acute leukemia in patients with polycythemia vera?

A

It is always a risk, but the disease can be controlled well for decades.

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21
Q

What are the two main types of treatment for Polycythemia Vera?

A

Phlebotomy and cytoreductive therapy.

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22
Q

What is the chance of polycythemia vera transforming into myelofibrosis?

A

About 10-30% after 10-20 years.

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23
Q

What is the role of daily aspirin in the treatment of Polycythemia Vera?

A

To help prevent thrombotic events.

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24
Q

What is essential thrombocytosis also known as?

A

Hemorrhagic thrombocytosis, idiopathic thrombocytosis, or primary thrombocytosis.

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25
Q

What are some complications of Polycythemia Vera?

A

Thrombosis, systemic hypertension, hemorrhage, organomegaly, pulmonary hypertension, pruritus, acid-peptic disease, erythromelalgia, hyperuricemia, gout, renal stones, myelofibrosis, acute leukemia.

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26
Q

What is the gender ratio for essential thrombocytosis?

A

More females are affected than males, with a 2:1 ratio.

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27
Q

What mutation is less common but still present in about 50% of essential thrombocytosis cases?

A

JAK-2 mutation.

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28
Q

What are some management strategies for complications of Polycythemia Vera?

A

Phlebotomy, antihistamines, aspirin, anagrelide, hydroxyurea, pegylated interferon, psoralens and ultraviolet-A light, coumadin, epsilon aminocaproic acid, allopurinol, thalidomide, imatinib, splenectomy.

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29
Q

What are common symptoms of essential thrombocytosis?

A

Thrombosis, easy bruising, erythromelalgia, headaches, visual changes, TIA/stroke, syncope.

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30
Q

What is the primary method for diagnosing essential thrombocytosis?

A

Diagnosis of exclusion and bone marrow biopsy revealing megakaryocyte hyperplasia.

31
Q

What are some causes of thrombocytosis?

A

Tissue inflammation, malignancy, infection, myeloproliferative disorders, myelodysplastic disorders, postsplenectomy, hemorrhage, iron deficiency anemia, surgery, rebound, hemolysis, familial causes.

32
Q

What are complications of essential thrombocytosis?

A

Microvascular ischemia, macrovascular thrombosis, hemorrhage due to acquired von Willebrand disease, transformation to acute leukemia.

33
Q

What are the primary types of leukemia mentioned?

A

Acute leukemia, chronic myelogenous leukemia, and hairy cell leukemia.

34
Q

What is the impact of essential thrombocytosis on survival?

A

The transformation to high risk does not impact overall survival.

35
Q

What are some non-malignant causes of myelofibrosis?

A

HIV infection, hyperparathyroidism, renal osteodystrophy, systemic lupus erythematosus, tuberculosis, vitamin D deficiency, thorium dioxide exposure, gray platelet syndrome, thrombopoietin receptor agonists.

36
Q

What are common symptoms of myelofibrosis?

A

Progressive fatigue, dyspnea, weight loss, night sweats, bleeding/bruising, bone pain, fevers.

37
Q

What is the primary focus of risk stratification in myelofibrosis according to MIPSS?

A

Incorporating mutational data.

38
Q

What are the key variables associated with reduced overall survival in myelofibrosis?

A

Hemoglobin < 100 g/L, peripheral blood blasts ≥2%, constitutional symptoms, absence of CALR type 1, high molecular risk mutations, unfavorable karyotype.

39
Q

How is treatment for myelofibrosis determined?

A

Based on risk and related symptoms/signs.

40
Q

What are the treatment options for lower-risk myelofibrosis patients?

A

Allogeneic HSCT, observation, or clinical trial.

41
Q

What are the treatment options for higher-risk myelofibrosis patients?

A

Anemia therapy, clinical trial, ruxolitinib, fedratinib, pacritinib, peginterferon alfa-2a, hydroxyurea.

42
Q

What is the role of JAK inhibitors in myelofibrosis treatment?

A

They are not selective for mutated JAK2 V617F protein.

43
Q

What is the hallmark clinical feature of myelofibrosis?

A

Splenomegaly.

44
Q

What is the recommended treatment for low-risk primary myelofibrosis patients under age 45?

A

Marrow transplantation if a matched sibling donor is available.

45
Q

What imaging techniques are important for diagnosing splenomegaly in myelofibrosis?

A

Abdominal ultrasound or CT.

46
Q

What is the treatment approach for asymptomatic thrombocytosis in essential thrombocytosis?

A

No therapy is required in the absence of thrombotic or significant hemorrhagic diathesis.

47
Q

What is the preferred treatment for erythromelalgia in essential thrombocytosis?

A

Aspirin, unless ristocetin cofactor activity is reduced.

48
Q

What is primary myelofibrosis?

A

A clonal stem cell disorder with abnormal, excessive marrow fibrosis and extramedullary hematopoiesis.

49
Q

What is secondary myelofibrosis?

A

It can result from other illnesses like polycythemia vera, CML, or MDS with fibrosis.

50
Q

What is the recommended treatment for high-risk primary myelofibrosis patients up to age 65?

A

Reduced-intensity conditioning marrow transplantation.

51
Q

What are the major criteria for diagnosing primary myelofibrosis according to WHO?

A

Megakaryocytic proliferation and atypia, JAK2, CALR, or MPL mutation, and not meeting criteria for other myeloid malignancies.

52
Q

What condition was diagnosed in the 55-year-old woman after her biopsy?

A

Breast cancer (Ductal Carcinoma In-Situ)

53
Q

What are some complications associated with primary myelofibrosis?

A

Anemia, thrombocytopenia, splenomegaly, portal hypertension, pulmonary hypertension, organ compromise, bone marrow failure, acute leukemia.

54
Q

What are the treatment options for symptomatic anemia in primary myelofibrosis?

A

Corticosteroids, recombinant erythropoietin, folate, danazol, low-dose thalidomide, targeted JAK2 inhibition.

55
Q

What abnormal blood values were found in the woman’s pre-operative blood work?

A

Elevated WBC, Hgb, Hct, and Plts; JAK2 positive; low EPO

56
Q

What are the treatment options for splenomegaly in primary myelofibrosis?

A

Targeted JAK2 inhibition, low-dose interferon, low-dose thalidomide and prednisone, hydroxyurea.

57
Q

What factors worsen the prognosis of primary myelofibrosis?

A

Hemoglobin < 10 g/dL, white cell count <4,000 uL or > 30,000, age >65, abnormal karyotype, circulating blasts > 1 percent, platelets < 100,000, leukemic transformation.

58
Q

What physical symptoms did the woman exhibit during her examination?

A

Ruddy cheeks, flushed facial appearance, palpable splenic tip

59
Q

What are the high molecular risk mutations in primary myelofibrosis?

A

IDH1/2, EZH2, ASXL1, SRSF2.

60
Q

What is the role of splenic irradiation and splenectomy in primary myelofibrosis?

A

Both are palliative and associated with significant risks.

61
Q

What was the initial treatment plan for the woman’s diagnosed condition?

A

Phlebotomy, frequent CBC monitoring, oral cytoreductive medication, Hydroxyurea, Aspirin

62
Q

What are the risk factors used in the clinicohematologic-based prognostic models of myelofibrosis?

A

Anemia (Hgb < 10 g/dL), constitutional symptoms, peripheral blasts > 1%.

63
Q

Who should be considered for allogeneic stem cell transplantation in primary myelofibrosis?

A

Younger patients with expected survival < 5 years, generally intermediate-2 or high-risk patients.

64
Q

What condition was the 73-year-old man diagnosed with in 1995?

A

Polycythemia Vera (P. Vera)

65
Q

What significant changes were observed in the man’s spleen over time?

A

Marked splenomegaly, spleen size increased significantly

66
Q

What are the survival rates for allogeneic stem cell transplantation in primary myelofibrosis?

A

1-year NRM rate: 12% (matched donors) to 38% (mismatched); 5-year survival rate: 56% (matched sibling donors) to 34% (partially matched/mismatched).

67
Q

What is the major cause of mortality in polycythemia vera (PV)?

A

Thrombosis.

68
Q

What was the man’s bone marrow biopsy result in 2013?

A

Persistent JAK2+ MPN with increased myelofibrosis, significantly more reticulin fibrosis, hypercellular marrow

69
Q

What were the main symptoms and complications the man experienced with his condition?

A

Itching, fatigue, oral ulcers, increasing splenomegaly

70
Q

What is the focus of therapy for essential thrombocythemia (ET) and polycythemia vera (PV)?

A

Thrombohemorrhagic risk reduction.

71
Q

What is the focus of therapy for myelofibrosis (MF)?

A

Individualized to address symptom/spleen reduction, anemia amelioration, and cure.

72
Q

What treatment options were discussed for the man’s condition progression to myelofibrosis?

A

Hydroxyurea, Jakafi, Interferon, continued phlebotomy, Plavix or Aspirin

73
Q

What should be considered for patients with myelofibrosis experiencing ruxolitinib failure?

A

Experimental options.

Hematology/Immunology Part 2 (9 decks)

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