Heme Flashcards
1
Q
Aplastic anemia
A
PIG-A ATE BCORL1 and BCOR
2
Q
Dyskeratosis congenita
A
DKC1 gene, pancytopenia by 10, reticulated hyperpigmentation, dystrophic nails and oral leukoplakia.
3
Q
Diamond Blackfan
A
Macrocytic anemia, short stature, abnormal thumb, craniofacial abnormalities, cardiac and urogenital abnormalities. RPS19 (ribosomal protein S19)
4
Q
ALK
IGH
MYC
A
2
14
8
5
Q
CLTC (clarithrin)
BCL2
MALT1
A
17
18
18
6
Q
Angioimmunoblastic T cell lymphoma
A
- High branching endothelial vessels
- T helper cell IHC (CD10, CD279, BCL6, CXCL13, CXCR5, ICOS).
- FDC IHC is disrupted.
- Trisomy 3, 5 and additional X chromosome.
- EBV positive
7
Q
Anaplastic Large Cell Lymphoma-ALK positive and Negative (systemic)
A
- Bimodal (positive in children and negative in adults)
- Types are lymphohistiocytic, small cell and Hodgkin
- Negative has DUSP22, TP63, triple negative.
- IHC always CD30 positive with both cytoplasmic and golgi staining.
8
Q
Extranodal NK/T-cell lymphoma, nasal type
A
- NK cell IHC CD2, CD56 positive and CD3 negative with variable CD5 and CD7.
- T-cell IHC is CD2, CD3, CD56 positive or negative
- NK cell no clonal TCR rearrangement and T-cell has clonal rearrangement.
- Cells are angiocentric, angioinvasive and angiodestructive features.
- EBV positive.
9
Q
Subcutaneous Panniculitis like T-cell lymphoma
A
- Young adults with erythematous subcutaneous nodules or plaques.
- Hematophagocytic syndrome ( hepatosplenomegaly, cytopenia and hypertriglyceridemia)
- IHC is cytotoxic: CD2, CD3, CD5, CD7, CD8, TIA-1, granenzyme B and perforin.
10
Q
Hepatosplenic T-cell lymphoma
A
- Mostly gamma delta.
- Associated with thiopurine and infliximab.
- IHC: have non activated cytotoxic proteins like TIA-1 and granzyme M.
11
Q
Intestinal T-cell lymphoma
A
- EATL associated with celiac
- MEITL (monomorphic epitheliotrophic intestinal T lymphoma)
- Difference is CD8, CD56 and MATK positive in MEITL.
- EATL: gains of 1q and 5q
- MEITL: MYC
