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93 Cards in this Set
- Front
- Back
What other tyrosine kinase-activating mutation than JAK2 V617F can be found in some cases of polycythemia vera, and perhaps some other MPNs?
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JAK2 exon 12 mutation
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Systemic mastocytosis is often associated with what somatic mutations?
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c-kit
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In what percent of CML cases is the Philadelphia chromosome found? In the remaining cases, how is the characteristic transcript found?
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95% of CML cases are BCR-ABL positive. The remainder have to be picked up by FISH or RT-PCR. Prognosis is the same for Ph+ and Ph- CML.
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What is the significance of major breakpoint cluster region mutations in CML?
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This is typical CML, and the transcript is called p210BCR-ABL
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What is the significance of the micro-BCR p230BCR-ABL protein?
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Found in rare CML variant cases with chronic neutrophilia +/- thrombocytosis. The course is more indolent than typical CML.
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What is a Pseudo-Gaucher cell?
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Pseudo-Gaucher cells, which are found in many hematologic abnormalities, including CML, AML, CLL, Hodgkin lymphoma, multiple myeloma, and ITP, cannot be distinguished from Gaucher cells on H&E stained sections. However, Gaucher cells show diffuse iron staining, while pseudo-Gaucher histiocytes are generally negative.4 In pseudo-Gaucher cells, cellular debris accumulates when the capacity of lysosomes is overwhelmed by the material produced from apoptosing cells.
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What are sea-blue histiocytes?
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Marcophages that accumulate ceroids and/or lipofuscin in their cytoplasm. They are associated with a high rate of intramedullary cell death or storage diseases. Sea-blue histiocytosis can be observed in patients with genetic lipid metabolic diseases or ceroid storage diseases.
This morphological finding can be associated both with acquired conditions of increased cellular turnover and inborn errors of lipid metabolism |
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What is the significance of the p190BCR-ABL product in CML?
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Most often found in Ph-positive ALL. It also can be coexpressed with p210 or detected alone in cases associated with monocytosis.
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Why is RT-PCR preferred for monitoring disease response in CML?
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FISH for BCR-ABL has a 3% false positive rate
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What other TK activities are affected by imatinib (other than BCR-ABL)
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ARG, PDGFRA, PDGFRB, c-kit
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What is the most common mechanism of resistance to imatinib?
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50-90% due to point mutations that affect the TK domain of ABL (the site of imitinib binding)
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What are the significant differences between dasatinib and nilotinib?
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Dasatinib has no structural similarity to imatinib, whereas nilotinib is an imatinib-derivative with 30-fold greater potency.
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What is the significance of T315I in CML?
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It's called the gatekeeper mutation, and is resistant to all 3 TKi's.
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How should CML patients with myeloid or lymphoid blast crisis be managed after acheiving hematologic response on second generation TKi's.
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Allogeneic HSCT, until long term data on the second generation drugs are out.
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What is the definition of accelerated phase in CML?
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•10 to 19 percent blasts in the peripheral blood or bone marrow
•Peripheral blood basophils ≥20 percent •Platelets <100,000/microL, unrelated to therapy •Platelets >1,000,000/microL, unresponsive to therapy •Progressive splenomegaly and increasing white cell count, unresponsive to therapy •Cytogenetic evolution (defined as the development of chromosomal abnormalities in addition to the Philadelphia chromosome) |
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What is the definition of blast crisis in CML?
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•≥20 percent peripheral blood or bone marrow blasts [3]
•Large foci or clusters of blasts on the bone marrow biopsy [4] •Presence of extramedullary blastic infiltrates (eg, myeloid sarcoma also known as granulocytic sarcoma or chloroma) |
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Which type of CML blast crisis is more likely to respond to chemotherapy (achieve a second chronic phase) AML or ALL?
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With myeloid blast crisis, induction
chemotherapy regimens used for acute myeloid leukemia (AML) can achieve a second chronic phase in 20% to 30% of patients, whereas regimens for ALL are effective in 40% to 60% of cases with lymphoid blast crisis. |
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What is the response rate, 5-year PFS and OS rate to front-line TKi therapy in CML?
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Currently, the CCyR rate to frontline
TK inhibitors is 70% to 90%, with 5-year progressionfree survival and overall survival rates between 80% and 95%. |
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What are the milestones that support the likelihood of achieving a good outcome in CML?
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• Achieving CHR at 3 months
• Achieving at least an MCyR at 12 months • Achieving CCyR at 18 months |
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Do CML patients always follow the chronic - accelerated - blast course?
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Blast crisis may develop suddenly, without
an intervening accelerated phase, in up to one fourth of chronic-phase patients. By comparison, a sudden transformation from chronic phase to blast phase occurs in 5% of patients on IFN therapy. Sudden transformation to blast phase in patients taking imatinib is observed with an annual rate of 1% to 2%. |
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What are the characteristic cutaneous findings in systemic mastocytosis?
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Cutaneous manifestations of mastocytosis typically include
a reddish-brown maculopapular eruption (urticaria pigmentosa) or, less often, a diffuse erythema, plaques, nodules, or the classic description of urticaria following stroking of the skin (Darier sign). |
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What are the first-line treatments in cutaneous mastocytosis?
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Treatment of CM includes H1 and H2 antihistamines, cromolyn
and other mast cell stabilizers, topical or intralesional glucocorticoids, and psoralen and ultraviolet A (PUVA) phototherapy. |
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Why are opiod analgesics a problem in patients with mastocytosis?
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They are known mast cell degranulators and may produce severe
adverse reactions in sensitive individuals |
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What is the treatment approach for patients with systemic mastocytosis with and without bone complications?
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IFN can be helpful for patients with painful skeletal lesions
or mast cell tumors that threaten bony integrity. Corticosteroids are generally avoided in this case because of their potential adverse effects on bone density. Patients with evidence of end-organ damage without major bony complications should receive a combination of corticosteroids and IFN. |
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What single-agent chemotherapy regimen is standard in patients with symptomatic systemic mastocytosis?
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Single-agent cladribine, given
as 5-day treatment cycles every 4 to 6 weeks |
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What is the role of imatinib in treatment of systemic mastocytosis?
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Some patients without the D816V mutation and with ASM, MCL, or SM-AHNMD may respond to imatinib
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How common is transition from cutaneous to systemic mastocytosis?
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Rare. Also, cutaneous involvement in SM appears to confer an indolent
behavior |
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What are the demographic characteristics that are associated with PDGFRA-related neoplasms?
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considerably more common in men (male-tofemale
ratio, 9:1 to 17:1) and are usually diagnosed between the ages of 25 and 55 years (median age of onset is late 40s). PDGFRA-related neoplasms seem to represent approximately 50% of patients with HES |
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What is the most serious complication of PDGRFRA-related neoplasms?
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endomyocardial fibrosis with ensuing restrictive cardiomyopathy
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What is the fusion gene in PDGFRA-related neoplasms?
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FIP1L1-PDGFRA. FISH testing relies on the probe for
the CHIC2 gene, which is uniformly deleted in patients with the FIP1L1-PDGFRA fusion gene. RT-PCR can be used both for diagnosis and monitoring of disease response and for minimal residual disease monitoring |
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What are the cell surface markers for activated eosinophils?
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CD23, CD25, CD69
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What is the treatment and prognosis for patients with PDGFRA-related neoplasms?
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Treatment is with imatinib, which is extremely effective. 5-year survival is now ~80%.
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What is the usual presentation of PDGFRB-related neoplasms?
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Patients with PDGFRB-related neoplasms tend
to present with features characteristic of chronic myelomonocytic leukemia with associated eosinophilia |
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What is the fusion gene product in PDGFRB-related neoplasms?
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t(5;12) translocation involving ETV6(12p13) and
PDGFRB (5q33) |
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What is the treatment and prognosis for PDGFRB-related neoplasms?
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Treatment is imatinib. Although there are only
small case series on the impact of imatinib on the survival of patients with PDGFRB-related neoplasms, these studies suggest a similar benefi t to that seen in patients with PDGFRArelated neoplasms, with median survivals exceeding 5 years. |
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What are the 3 different ways in which FGFR1-related neoplasms can present?
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FGFR1-related
neoplasms can present as classic MPNs, precursor B- or T-cell lymphoblastic leukemia, or AML. Median age is 32. |
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What is the most common genetic aberration in FGFR1-related neoplasms?
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The
most common chromosomal translocation associated with FGFR1-related neoplasms is t(8;13)(p11;q12), which results in expression of the ZNF198-FGFR1 fusion TK. |
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What is the treatment and prognosis of FGFR1-related neoplasms?
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Early intensive therapy followed by allogeneic SCT remains
the only potential curative therapy for patients with FGFR1- |
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What is required for diagnosis of chronic eosinophilic leukemia-NOS?
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The revised 2008 WHO criteria
also require the presence of eosinophilia (1.5 109/L); presence of clonal cytogenetic or molecular abnormality or blasts cells 2% in the peripheral blood or 5% in the bone marrow; lack of BCR-ABL1, PDGFRA/PDGFRB, or FGFR1 rearrangements; bone marrow blasts 20%; and absence of inv(16)(p13.1q22). |
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What are the four body systems affected by CES-NOS other than the heart?
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Peripheral and central nervous system fi ndings
can include mononeuritis multiplex, peripheral neuropathy, and paraparesis, as well as cerebellar involvement, epilepsy, dementia, cerebral infarction, and eosinophilic meningitis. Pulmonary involvement includes idiopathic infi ltrates, fi brosis, pulmonary effusions, and pulmonary emboli. Skin manifestations are common and can take many forms, including angioedema, urticaria, papulonodular lesions, and erythematous plaques. Gastrointestinal involvement by eosinophilia can result in ascites, diarrhea, gastritis, colitis, pancreatitis, cholangitis, or hepatitis |
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What is the treatment approach to CES-NOS?
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First line = steroids, then hydroxyurea
Second line = vincristine-etoposide Also consider anti-IL-5 antibody for rapid response. Campath has been reported successful in 2 case reports. |
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What is the genetic aberration in PV? In what percentage of cases is it found?
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JAK2 V617F is found in 90-95% of cases.
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What is the most common genetic aberration in PV patients who are JAK2 V617F-negative?
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JAK2 exon 12 mutations are specifi c to JAK2
V617F–negative PV and are most often identified in patients with erythrocytosis without associated thrombocytosis or leukocytosis. |
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What are the granulocyte and platelet abnormalities associated with PV?
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Qualitative platelet defects including acquired vWF disease, and inappropriate granulocyte activation.
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How can Budd-Chiari syndrome mask a diagnosis of PV?
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Liver dysfunction can lead to fluid overload, which causes a normal hematocrit despite an elevated red cell mass. However, in the acute setting, hepatic necrosis can be
associated with transiently elevated EPO levels, misleadingly suggesting a secondary, EPO-driven erythrocytosis |
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In addition to kidney and liver tumors, what other tumors can produce EPO?
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cerebellar hemangiomas, uterine
myomas, ovarian tumors, parotid tumors, lymphomas, and adrenal tumors can be associated with pathologic EPO production. |
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Is there a role for phlebotomy in secondary polycythemia?
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In secondary polycythemia, phlebotomy
may occasionally be indicated to decrease blood viscosity and improve oxygenation when symptoms occur or prophylactically, especially when hematocrit values exceed 60%. |
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Describe the types of "ambiguous" cases related to possible PV.
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10-40% of patients with an elevated RCM, normal or near-normal EPO level and no obvious cause for secondary polycythemia will go on to have features of PV, while most cases will spontaneously resolve. Some JAK2 exon 12 positive patients will have idiopathic erythrocytosis that persists for years without progressing.
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What labs are helpful in diagnosing JAK2-negative PV patients?
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Elevated LAP score, elevated B12 level, elevated LDH, elevated uric acid, EEC growth, PRV1
granulocyte overexpression, decreased c-Mpl expression, marrow hypercellularity with megakaryocyte clustering |
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What is the prognosis for patients with polycythemia vera?
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Once the diagnosis is secure, treatment with phlebotomy,
with aspirin, and with or without cytoreductive agents results in a near-normal life span for the average patient who is diagnosed at a median of 60 to 65 years of age. However, although it exceeds 10 years, the median survival of individuals diagnosed with PV before 40 years of age is considerably shorter than the life expectancy for a healthy person of similar age, even with active management |
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Describe postpolycythemic myelofibrosis. What is the incidence of AML in these patients?
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Postpolycythemic myelofi brosis is characterized by
progressive hepatosplenomegaly due to extramedullary hematopoiesis, advanced marrow fi brosis, and pancytopenia with leukoerythroblastosis. The median survival time in patients with postpolycythemic myelofi brosis is 3 years. At least 25% to 50% of patients with postpolycythemic myelofi brosis develop AML, although it is important to note that not all PV patients who transform to AML will progress through a postpolycythemic myelofi brosis phase. |
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Which PV therapies are leukemogenic and which are not?
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Phosphorus-32
(32P) treatment, chlorambucil, busulfan, and alkylating agent combinations are associated with up to 15-fold increase in transformation to AML. Hydroxyurea, IFNa and anagrelide are not leukemogenic. |
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How should patients with PV be managed in a perioperative setting?
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Up to 80% of patients with PV who undergo
surgery in the context of a hematocrit and/or platelet count markedly above the normal range will suffer a thrombohemorrhagic event. The hematocrit and platelet count should be normalized for several weeks prior to elective surgery to minimize the risk of perioperative thrombosis and bleeding in patients with PV. Postoperative thrombosis prophylaxis should be given whenever possible. |
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What are the phlebotomy goals for patients with PV?
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Generally accepted goals include a hematocrit of 45% for
men, 42% for women, and 37% for pregnant women late in gestation |
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When should immunosupressive therapy accompany phlebotomy in PV?
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Aggressive initial phlebotomy without adjunctive myelosuppressive therapy may be associated
with a higher risk of thrombosis, particularly in the elderly and those with a prior history of thrombosis; this has been hypothesized to be the result of new platelet formation in response to the thrombopoietic stimulus of rapid phlebotomy. Based on these clinical observations, myelosuppressive therapy is recommended as part of the initial treatment of patients 60 years of age and younger patients with thrombotic risk factors, particularly a history of thrombosis |
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How should high-risk women of childbearing age be treated for PV?
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IFNa
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Describe the ECLAP trial as relates to the treatment of PV.
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The ECLAP study, a double-blind randomized trial, compared
low-dose aspirin with placebo among 518 patients who had no indication for anticoagulation and no preexisting clear indication or contraindication to aspirin therapy and demonstrated that low-dose aspirin (eg, 100 mg/d) reduces the rate of thrombosis and cardiovascular deaths in patients with PV receiving standard phlebotomy and supportive care. The aspirin-treated group suffered 60% fewer major thromboses and cardiovascular deaths |
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How is PV-related pruritis (unresponsive to phlebotomy or hydroxyurea) managed? Painful splenomegaly and/or hypercatabolic symptoms?
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Antihistamines, PUVA, cholestyramine, or selective
serotonin reuptake inhibitors (eg, paroxetine) may provide symptomatic relief. Cytoreductive therapy with hydroxyurea or IFN may help in refractory cases. Painful splenomegaly and unacceptable hypercatabolic symptoms also usually require treatment with hydroxyurea or IFN. Splenectomy or splenic irradiation may be necessary for palliation in selected patients who are intolerant of or unresponsive to cytoreductive agents. |
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In addition to talking to patients about the (at least) hypothetical increase in risk for AML, what other adverse effects of hydroxyurea should be discussed?
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Cytopenias
and, less commonly, chronic mucocutaneous ulcers |
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What are the main adverse effects of anagrelide?
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Anagrelide is a vasodilator; adverse effects, including headache, palpitations, diarrhea,
and fl uid retention, may be avoided or minimized by starting at a lower dose and titrating up. |
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What is the role of Phosphorous-32 in treatment of PV?
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32P has been demonstrated in randomized
trials to increase the risk of hematologic and nonhematologic malignancies, particularly when combined with alkylating agents or hydroxyurea. However, it is well tolerated in the short term, and responses after a single treatment may last several months. Recent studies indicate that lower-dose 32P may be equally effective as conventional doses, but with reduced and delayed risk of malignancy development. Therefore, low-dose 32P is a reasonable palliative option for patients 70 years of age and may be especially useful in patients whose blood counts are diffi cult to control with hydroxyurea. |
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What roles does HSCT have in treatment of PV?
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Currently, the high risks of morbidity and mortality
with myeloablative allogeneic transplantation and the baseline favorable prognosis with PV restrict the use of SCT to younger patients with a poor prognosis |
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What is the difference between the two general types of familial polycythemic states?
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They can be either truncating EPO receptor
mutations (low endogenous EPO level) or VHL/HIF2A mutations (high endogenous EPO level). |
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What percentage of patients with ET are JAK2 positive, and what are the clinical implications for these patients?
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Patients with
ET who have JAK2 V617F have a higher median hemoglobin concentration and neutrophil count than those who lack the mutation, and they may have more thromboembolic events and require more cytoreductive therapy |
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How are ET and PV different in terms of presenting signs and symptoms?
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hypercatabolic signs and constitutional symptoms
are uncommon in ET |
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Which hematologic malignancies have to be excluded before making a diagnosis of ET?
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MPNs (especially PV and CML) and MDS [especially 5q–
syndrome, chromosome 3(q21;q26) abnormalities, or refractory anemia with ringed sideroblasts associated with marked thrombocytosis (RARS-T)]. |
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How does the LAP score help sort various MPNs?
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The LAP score is typically elevated in ET, like PV but unlike
CML |
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What are the causes of Howell-Jolly bodies?
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Common causes of asplenia are splenectomy following trauma to the spleen, and autosplenectomy caused by sickle cell anemia. Ten percent of patients with Coeliac disease also present with splenic atrophy with subsequent Howell-Jolly bodies. Other causes are radiation therapy involving the spleen, such as that used to treat Hodgkin lymphoma. Howell-Jolly bodies are also seen in: severe hemolytic anemia, megaloblastic anemia, hereditary spherocytosis, and myelodysplastic syndrome (MDS).
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What is the general prognosis for patients with ET?
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Life expectancy in the first decade after diagnosis is similar to age-matched controls. Transformation to AML is also rare in the first decade but increases after 2-3 decades.
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How does platelet count relate to risk of thrombotic or hemorrhagic complications in ET?
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Although some studies have
found an increased risk of bleeding in ET patients with a platelet count of 1,500,000/L, there are no data to suggest that absolute platelet number is clearly predictive of thromboembolic complications in the absence of other clinical risk factors. Nevertheless, controlling the platelet count may be protective, and studies with hydroxyurea (targeted at maintaining the platelet count at 600,000/L) and anagrelide (targeted at maintaining the platelet count at 400,000/L) have shown that these drugs decrease the risk of primary or recurrent thrombotic events (from 24% to 3.6% during the 27-month observation period). |
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What are the two major risk factors in ET, and how should this group be treated?
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Age > 60 and/or history of thromboembolic event = high risk.
Patients with ET at high risk of thrombosis should be treated with low-dose aspirin and hydroxyurea with a goal platelet count of 400,000 to 600,000/L. Anagrelide should be reserved for patients with an insuffi cient response to hydroxyurea or with intolerable hydroxyureaassociated adverse effects. |
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How is treatment of pregnant patients, or patients who desire pregnancy, different between PV and ET?
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It isn't. Both use IFNa, either standard or pegylated form.
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What is the general treatment approach in terms of thromboemolism prophylaxis in ET?
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Platelet-lowering agents and low-dose aspirin are indicated
for high-risk patients with ET. Hydroxyurea should be the fi rst choice because it is superior to anagrelide at preventing most thrombohemorrhagic events. Anagrelide can be a useful second-line agent. Low-dose aspirin alone should be used for low-risk patients and may effectively treat vasomotor symptoms. |
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Which cell surface marker has some prognostic value in PMF?
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The number of circulating CD34
cells in PMF patients can be 50-fold higher than in PV or ET. Higher levels of circulating CD34 cells in PMF are associated with more advanced bone marrow fi brosis and other disease characteristics. One study observed shorter survival and earlier transformation to AML among PMF patients with circulating CD34 cell counts >300/microL |
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What are the two hallmarks of PMF?
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marrow fi brosis and extramedullary
hematopoiesis; |
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What percentage of patients with PMF are JAK2 positive?
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50%
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In JAK2 V617F-negative PMF and ET patients, what mutation can activate JAK2 in up to 5-10% of patients?
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MPL
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What are the general requirements for diagnosing PMF (not the acutal diagnostic criteria)?
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increased bone marrow
reticulin or collagen fi brosis, leukoerythroblastic peripheral blood fi ndings, and splenomegaly, in the absence of a secondary cause for these fi ndings or of features better fitting ET or PV or CML |
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How can certain autoimmune conditions mimic PMF?
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Secondary marrow changes of increased reticulin
fibrosis with megakaryocytic hyperplasia resembling PMF have been associated with systemic lupus erythematosus, Sjögren syndrome, psoriatic arthritis, and other chronic autoimmune diseases, most commonly in the absence of splenomegaly or signifi cant leukoerythroblastic peripheral blood findings |
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What are the four features of the leukoerythroblastic peripheral blood smear?
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immature myeloid cells,
nucleated red blood cells, teardrop erythrocytes, and large platelets. |
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Describe the prefibrotic or "cellular" stage of PMF.
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Approximately 20% to 30% of patients with PMF are
believed to present in the prefi brotic stage. The early prefi - brotic stage of PMF, also referred to as the “cellular” or “proliferative” stage, may be associated with thrombocytosis and modest leukoerythroblastosis but may be diffi cult to distinguish from ET. This condition may cause diagnostic diffi culties in the absence of clear evidence of megakaryocyte atypia on bone marrow biopsy |
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Risk of developing AML is increased in PMF patients with what hematologic abnormalities?
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Severe anemia and high number of circulating immature myeloid cells.
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What is the treatment of choice for PMF patients with hypercatabolic symptoms, painful splenomegaly, leukocytosis or thrombocytosis?
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Hydroxyurea.
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Which patients with PMH are most likely to respond to therapy with EPO?
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Those with endogenous EPO level of < 120 who have not yet progressed to transfusion dependence.
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How is thalidomide used in PMF?
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Low-dose (50 mg/d), with or without a tapering dose of oral prednisone
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How is PMF-associated hemolytic anemia treated?
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May rarely respond to
corticosteroids, danazol, or cyclophosphamide |
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What is the role of splenectomy in treatment of PMF?
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Splenectomy is indicated for
palliation of portal hypertension, refractory anemia, and symptoms not controlled by cytotoxic agents. With the conventional laparotomy approach in experienced centers, postoperative mortality is approximately 10%, and the median survival is approximately 1 to 2 years |
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What are the risk factors for post-splenectomy thrombosis in PMF patients, how is this managed, and how is post-splenectomy hepatomegaly addressed?
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Patients at increased risk of thrombocytosis
are those with preoperative platelet counts 50,000 to 100,000/L; 18% to 50% of such patients will achieve postoperative levels 600,000/L. In this setting, hydroxyurea or anagrelide should be started or the dose increased the moment the postoperative platelet count exceeds the normal range. Massive hepatomegaly resulting from accelerated extramedullary hematopoiesis develops in 16% to 24% of patients after splenectomy. This complication may be diffi cult to control but may respond to cladribine |
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What is the role of radiation therapy in treatment of PMF?
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Irradiation can effectively palliate pain in 90% of
patients, and treatment can be repeated if necessary. Benefits may last several months, but irradiation carries a substantial risk of severe, prolonged cytopenias, which cannot be predicted by baseline blood counts or by the dose of radiation. Local irradiation can be effective for palliation of painful or threatening focal areas of extramedullary hematopoiesis (eg, with spinal or retroperitoneal myeloid tumors). |
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What is the role of HSCT in PMF?
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Myeloablative allogeneic SCT should be considered for
patients 55 years of age with poor prognostic features who have an HLA-compatible donor. Nonmyeloablative allogeneic SCT may benefi t selected patients who are older or who are not candidates for conventional SCT. |
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What is AHNMD?
|
Systemic mastocytosis (SM) is a myeloproliferative disease affecting multipotent and/or mast cell-committed hematopoietic progenitor cells. In a significant subgroup of patients (10–35%), an associated clonal hematologic non-mast cell lineage disorder (AHNMD) occurs. These AHNMDs can be classified according to recently established WHO criteria. Most AHNMDs resemble myeloid malignancies such as acute myeloid leukemia, myeloproliferative disorders or myelodysplastic syndromes. In only a few cases, lymphoproliferative disorders are diagnosed. Patients with SM-AHNMD have a less favorable prognosis concerning survival when compared to indolent SM. No general guidelines for the treatment of patients with SM-AHNMD have been established so far. A reasonable straightforward approach may be to treat the AHNMD in those patients in the same way as if no coexisting SM exists
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What are the cutoffs for CMML-1 and CMML-2?
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CMML-1 = <5% peripheral blasts and <10% bone marrow blasts.
CMM-2 = 5-19% peripheral blasts and 10-19% bone marrow blasts. |
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What enzyme can contribute to hypokalemia in CMML?
|
Muramidase/lysozyme can be a factor but does not account for all cases of hypokalemia in CMML.
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