Puthoff 4 Flashcards

1
Q

The diagnosis of AML is based on what?

A

Presence of at least 20% myeloid blasts in the BM

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2
Q

What is the difference between Myelodysplasic syndromes and myeloproliferative disorders?

A

In the dysplasias, there is ineffective hematopoises that leads to cytopenias
In the proliferative disorders, increased production of the mature cell

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3
Q

What are the 4 classes of AML determined by the WHO and which one has the best and worst prognosis generally?

A

AML with genetic mutations - best
AML with MDS like features
AML with therapy relation, so after getting chemo - worst
AMO, NOS

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4
Q

What is the class name and genetic aberration for M2, M3, M4 of the revised FAB classification of AML?

A

AML with myelocytic maturation 8:21
acute promyelocytic leukemia 15:17
AML with myelomonocytic maturation inversion of 16

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5
Q

Cd 34 and CD33 are markers for what cells?

A

Precursor immature myeloid progenitor

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6
Q

CD 64 is a marker for what cell?

A

Mature myeloid

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7
Q

6 Myelodysplastic syndromes he wants us to know?

A

RA, RARS, RAEB, RAEBt, CMML, MDS

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8
Q

What is the mutation in CML and what is the specific nick name of the mutation?

A

BCR-ABL fusion gene

Philadelphia chromosome

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9
Q

Big time clinical sign and lab sign of CML?

A

Massive spleen and leukocytosis

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10
Q

What must we rule out when a patient presents with Essential thrombocytosis? Also, what are the two mutations for ET?

A

A reactive reason why platelets are up like inflammation or iron deficiency
Jak 2 and MPL

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11
Q

What will we see on BM biopsy and a lab value for ET?

A

Large, atypical tons of Megas

Super high platelet counts, like million

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12
Q

What s the hallmark of primary myelofibrosis?

A

Obliterative bone marrow fibrosis by non neoplastic fibroblasts

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13
Q

What does the bone marrow fibrosis lead to?

A

Extramedullary HP in the spleen, so also big spleen

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14
Q

3 markers for langerhan cells?

A

S100+, cd1a+, and HLADR+

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15
Q

What are the 3 langerhan histiocytosis and their respective classification?

A
  1. Letterer siwe disease, multifocal
  2. Eosinophilic granuloma, mixed unifocal and multifocal
  3. Pulmonary langerhan, unisystem
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16
Q

What is the example of eosinophilic granuloma being multifocal?

A

Hand schuller christian triad: bone defects, diabetes insipidus, and exophthalmos

17
Q

What is the mutation for pulmonary langerhans and what is its High Yield association?

A

BRAF

Smoking