[醫如往常] Senior Clerkship Day 113, 115: Pathology: hematology

Hematology 林醫師
MDS; myelodysplastic syndrome
Pancytopenia
CD31 megaloblasts ↑
Erythroid aplasia
MPO<50%
CD34 ↑
CD117 ↑(membrane)
tdt↑(nuclear)
Ki-67↑


要配合Peripheral blood，MPN周邊血球是多而成熟，MDS是cytopenia
myeloproliferative neoplasms


Diagnostic criteria for MDS/MPN with ring sideroblasts and thrombocytosis

MDS/MPN diagnostic criteria

• Anemia associated with erythroid lineage dysplasia with or without multilineage dysplasia, ≥15% ring sideroblasts,* <1% blasts in PB and <5% blasts in the BM

• Persistent thrombocytosis with platelet count ≥450 × 109/L

• Presence of a SF3B1 mutation or, in the absence of SF3B1 mutation, no history of recent cytotoxic or growth factor therapy that could explain the myelodysplastic/myeloproliferative features†

• No BCR-ABL1 fusion gene, no rearrangement of PDGFRA, PDGFRB, or FGFR1; or PCM1-JAK2; no (3;3)(q21;q26), inv(3)(q21q26) or del(5q)‡

• No preceding history of MPN, MDS (except MDS-RS), or other type of MDS/MPN

• ↵* At least 15% ring sideroblasts required even if SF3B1 mutation is detected.
• ↵† A diagnosis of MDS/MPN-RS-T is strongly supported by the presence of SF3B1 mutation together with a mutation in JAK2 V617F, CALR, or MPL genes.
• ↵‡ In a case which otherwise fulfills the diagnostic criteria for MDS with isolated del(5q)-no or minimal absolute basophilia; basophils usually <2% of leukocytes.
來自 <http://www.bloodjournal.org/content/127/20/2391?sso-checked=true#T11>
------------
PB and BM findings and cytogenetics of MDS

Name	Dysplastic lineages	Cytopenias*	Ring sideroblasts as % of marrow erythroid elements	BM and PB blasts	Cytogenetics by conventional karyotype analysis
MDS with single lineage dysplasia (MDS-SLD)	1	1 or 2	<15%/<5%†	BM <5%, PB <1%, no Auer rods	Any, unless fulfills all criteria for MDS with isolated del(5q)
MDS with multilineage dysplasia (MDS-MLD)	2 or 3	1-3	<15%/<5%†	BM <5%, PB <1%, no Auer rods	Any, unless fulfills all criteria for MDS with isolated del(5q)
MDS with ring sideroblasts (MDS-RS)
MDS-RS with single lineage dysplasia (MDS-RS-SLD)	1	1 or 2	≥15%/≥5%†	BM <5%, PB <1%, no Auer rods	Any, unless fulfills all criteria for MDS with isolated del(5q)
MDS-RS with multilineage dysplasia (MDS-RS-MLD)	2 or 3	1-3	≥15%/≥5%†	BM <5%, PB <1%, no Auer rods	Any, unless fulfills all criteria for MDS with isolated del(5q)
MDS with isolated del(5q)	1-3	1-2	None or any	BM <5%, PB <1%, no Auer rods	del(5q) alone or with 1 additional abnormality except −7 or del(7q)
MDS with excess blasts (MDS-EB)
MDS-EB-1	0-3	1-3	None or any	BM 5%-9% or PB 2%-4%, no Auer rods	Any
MDS-EB-2	0-3	1-3	None or any	BM 10%-19% or PB 5%-19% or Auer rods	Any
MDS, unclassifiable (MDS-U)
with 1% blood blasts	1-3	1-3	None or any	BM <5%, PB = 1%,‡ no Auer rods	Any
with single lineage dysplasia and pancytopenia	1	3	None or any	BM <5%, PB <1%, no Auer rods	Any
based on defining cytogenetic abnormality	0	1-3	<15%§	BM <5%, PB <1%, no Auer rods	MDS-defining    abnormality
Refractory cytopenia of childhood	1-3	1-3	None	BM <5%, PB <2%	Any

• ↵* Cytopenias defined as: hemoglobin, <10 g/dL; platelet count, <100 × 109/L; and absolute neutrophil count, <1.8 × 109/L. Rarely, MDS may present with mild anemia or thrombocytopenia above these levels. PB monocytes must be <1 × 109/L
• ↵† If SF3B1 mutation is present.
• ↵‡ One percent PB blasts must be recorded on at least 2 separate occasions.
• ↵§ Cases with ≥15% ring sideroblasts by definition have significant erythroid dysplasia, and are classified as MDS-RS-SLD.


來自 <http://www.bloodjournal.org/content/127/20/2391?sso-checked=true#T15>




------------
Myeloma
CD138 plasma cell
CD56 +/-
CD79a -
------------
Megalokaryocytes: CD31


------------
Desmoplastic small round cell tumor
WT1 +
CK -
------------
Diffuse large B cell
Intra vascular
------------
尤醫師
Paracortex: T cells
Mantle zone: naïve B
Germinal center: hyperpigmentation, class swich
------------
Low grade B cell lymphoma: Congo red +
Follicular type: BCL6, CD10 (normal在neutrophil, stroma)
Mantle zone: cyclinD1, CD5 (normal在T-cell)
Marginal zone: CD43
CLL: CD5, CD23 (normal在follicular dendritic cells)
SLL
------------
 Chronic lymphocytic leukemia in (Richter's) transformation
出現平常B cell沒有的CD43
Germinal center 喪失bcl-2
------------
Clusters of CD123 positive PDC were observed in Kikuchi's disease
Post. neck
------------
Asia nose necrosis->NK/T cell lymphoma
Angiocentric pattern
CD3 (cyto)
CD5 (-)
------------