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T LBL+ AML (M6, myeloid Sa)+ CEL+ SM (reactive) (456616)
T LBL+ AML (M6, myeloid Sa)+ CEL+ SM (reactive)new
Subtitle: b12-38480		 Type:
HEMATO		 Sender:
ugnius 		2013-01-15 11:47
INCTR - EBMWG Hematopathology Online

62 yrs old male. ECOG 1.

 HISTORY: 2010 isolated erythrocytosis and eosinophilia 1.3. JAK2(-). No splenomegaly. Due to COPD reative erythrocytosis was diagnosed and 2 erythrophareses were done. 2012 maculopapular rush on the legs (photo macro below). Leukocytosis 12, eosinophilia 1.6. Thrombocythopenia 63. Anemia. PDGFRA and Bcr-abl1 (-). AML molecs (-). Next 6 months: leucocytosis 12-19L, eosinophilia 1,6-3,9, anemia ir thromboiytopenia. Peripheral blasts 0,5-1%. Hepato-splenomegaly and peripheral lymphadenopathy evolves (axillary up to 2cm). New rush elements on the hands, back abdomen with central necrosis. Blood: leucocytosis 19, Eosinophilia 3.97. RBC 4,5. HB-111 g/l. PLT-63. 

Episode 1 (2012 July):

A. Threpine biopsy: AML, M6+ SM (reactive?)+ CEL. 

B. Skin biopsy (not included): Dermatitis, drug reaction possible.

Episode 2 (2012 December):

A. Threpine biopsy: AML, M6+ SM (reactive?)+ CEL.

B. Skin biopsy: T pseudolymphoma with epidermothropism (mycosis like)(in the clinical cotext- if isolated: mycosis fungoides would be possible).

C. Node biopsy: Myeloid sarcoma (M6)+ T LBL. FLOW (diluted): disgranulopoiesis + eosinophilia. Without blastemia.

MOLEC's (aspirate/diluted): PDGFRA/FIP1 and Bcr-Abl1, JAK2 (-). Karyotype XY normal.

CLINICS: treatment: 3 weeks with Imatinib 1000mg/day. Eosinophilia diminished from 3.6 iki 2.9. Hb and PLT stable. Eruptions in hand's skin in resolution.

Proposal: NOT VERIFIED/POSSIBLE PDGFRA/PDGFRB/FGFR1 asociated multiple tumors: Myeloid sarcoma (AML M6 in BM)+ CEL+ SM (reactive?) (BM)+ T lymphoblastic lymphoma (node).

Thankyouforalldone.

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tzankov
2013-01-31 07:28
Challenging case. Altogether it looks like a haemto-lymhoid neoplasia associated with a putative thyrosine kinase gene rarranagement. Thus, molecular genetics is of central importance. Since the symptoms somehow responded to imatinib, FGFR1 is probably not the hottest candidate, but this is ony speculation. Would you more profoundly study the TdT+ population (CD1a, CD2, CD4, CD7, CD8, LMO2?). The mast cells are not the typical mast cells of SM; myeloid and lymphoid neoplasms with eosinophilia are often accompanied by mast cell proliferation distinct from SM. I am not sure that there is enough evidence to call what we see AML M6, I rather think that this is a part of the myeloid outgrowth of the neoplasm.
ugnius
2013-01-31 08:16
1. Thank you a lot. "M6 like" MPN was not treated as AML yet (I dont know how exactly we must call this extramyeloid blastic formally thing in the node).  
2. I will be beack with TdT+ LBL IH.  
3. Which techno you use for PDGFRA- B- FGFR (FISH based?)?  
We have PDGFRA PCR in clin lab only at the moment. It was negative, but I'm not quite sure about representativity of sample.
Last modified: 2013-02-28 06:46:14