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ONC Biweekly 2 > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

increased peripheral cell counts of granulocytes

A

Chronic myeloid leukemia

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2
Q

increased peripheral cell counts of platelets

A

essential thrombocytosis

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3
Q

increased peripheral cell counts of RBCs

A

Polycythemia vera

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4
Q

overproduction of fibrous tissue in bone marrow, leading to low peripheral cell counts

A

myelofibrosis

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5
Q

t(9;22) BCR-ABL

A

chronic myelogenous leukemia

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6
Q

JAK2 increased

A

Polycythemia vera
essential thrombocytosis
myelofibrosis

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7
Q

increased RBCs without high EPO
increased viscosity of blood

A

Polycythemia vera

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8
Q

Aquagenic pruritis (unbearable pruritis after warm bath or shower)

A

Polycythemia vera

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9
Q

associated with _____

A

Polycythemia Vera

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10
Q

increase in platelet count
must exclude reactive thrombocytosis

A

essential thrombocytosis

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11
Q

sx’s include bleeding and thrombosis

A

essential thrombocytosis

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12
Q

fibroblasts activated by PDGF and TGF-B (growth factors of megakaryocytes) and produce excess collagen in bone marrow

A

primary myelofibrosis

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13
Q

leads to marrow failure and then extramedullary hematopoisesis

A

primary myelofibrosis

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14
Q

clinical features of this include massive splenomegaly due to extramedullary hematopoisesis

A

primary myelofibrosis

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15
Q

tear drop cells are classic finding of _____

A

myelofibrosis

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16
Q
A

primary myelofibrosis

17
Q

rare
neoplastic proliferation of dendritic cells (cells of myeloid origin)
CD1a, S100, CD207

A

Langerhans cell histiocytosis

18
Q

birbeck granules found in cytoplasm of what

A

Langerhans cells

19
Q

children
any organ system
often bone and skin involvement

A

Langerhans cell histiocytosis

20
Q

most severe of the Langerhans cell histiocytosis
children
skin rash
bone lesions
fatal

A

Letterer-Siwe Disease

21
Q

least severe of the Langerhans cell histiocytosis
adolescents
BONE fracture (not osteosarcoma)
langerhans cells/eosinophils

A

Eosinophilic granuloma

22
Q

middle severity of the langerhans cell histiocytosis
TRIAD: scalp lesion, diabetes insipidus, exopthalmos

A

Hand-Schuller-Christian disease

23
Q
A

Hand-Schuller-Christian disease

24
Q

neoplastic proliferation of mast cells
all ages
cutaneous mastocytosis
systemic mastocytosis

A

mast cell neoplasms

25
Q
A

cutaneous mastocytosis

26
Q
A

cutaneous mastocytosis

27
Q

Mast cells are being produced in the bone marrow in the first place (systemic)
can be in other organs
Diaphoresis, weight loss, fever, night sweats, fatigue
Diarrhea (if GI involved)
KIT mutation

A

systemic mastocytosis

ONC Biweekly 2 (10 decks)

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