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BONE MARROW FAILURE: AA LIKE (817290)
BONE MARROW FAILURE: AA LIKEnew
Subtitle: b16-46314
Type:
HEMATO
Sender:
ugnius
2017-01-09 15:01
INCTR - EBMWG Hematopathology Online
CASE IS READY.  
 
55 years old women.  
 
STORY (from responsible clinician AD): 2013 referred to hematologist beacause of hemorrhagic rash on the legs and fever.  
The blood count showes neutropenia at 0,5x10*9/L, arregenerative normocytic anemia at 6,3 g/dl and thrombocitopenia at 12x10*9/L. No other concurrent disease, no history of medicine uptake. Cholecystectomic surgery at her age of 42.  
No history of blood transfusion.  
The clinical diagnosis of aplastic anemia was established. She had no HLA matched relative bone marrow donor, so immunosupressive therapy with rabbit ATG + cyclosporin was initiated with a resonse. The patient became independent of platelete and erythrocyte transfusion.  
2014 April the platelete count and hemoglobin dropped to transfusion level and second line therapy with hosre ATG + cyclosporin was realised with a temporal response.  
Thired line therapy with eltrombopag was started in JAN 2016. Patient became free of transfusion with plateletes at around 30-40x10*9/L, hemoglobin - 9-9,5 g/dl, neutrophiles - 1x10*9/L. The patient is on Exjade because of secondary hemochromatosis.  
 
MOLECS: cytogenetic analysis is performed every 6 months for possible clonal evolution and is negative. No positive molecular markers on SNP or NGS analysis.  
 
HISTO (several threpines eith similar changes): BM with FOCAL cellularity up to 60% (from 0%): dezorganized architecture: large, irregular erytroid diserythropoietic islands with immature precursors. M/E ratio inverted ~1/2. Medium large sized (NOT DYSPLASTIC TYPICALY) MEGAS with lobated nuclei and clustering up to 13 DPRL. Massive Fe deposition (posttransfusion). No prominent reticulin fibrosis, focal  
DIAGNOSTIC QUESTION: DEFINITE INTERPRETATION OF "MDS/AA LIKE" CHANGES IN BM: REGENERATING BM AFTER AA?  
Slight discordance with peripheral blood (never classical picture of AA was seen).  
 
Thankyouforbeeingtogether.  
 
 
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tzankov
2017-01-09 17:39
My diagnosis would be dysplastic and myeloproliferative-like bone marrow changes of unknown significane, probably related to the eltrombopag treatment (see also Am J Clin Pathol 2002;117:844)  
ugnius
2017-01-10 10:16
Thank You. Agree on "unclassifyiness". No MPN signs in blood and no mega dysplastic changes and no typical AA signs from beginning...
ugnius
2017-01-10 10:25
But still PRIMARY diagnosis left unresolved- I'll append 2013 and 2015 BMs.
tzankov
2017-01-10 13:09
has someone ever thought of a T-LGL? FACS? CD5 loss on CD3+ T-cells? CD57?  
I just stated "myeloproliferative-like bone marrow changes" to describe the grouped megakryopiesis and the hyperplastic erythdoid lineage
ugnius
2017-01-14 15:05
Pleas find 2013 and 2015 BMs: lower cellularity, diminished granulopoiesis, erythroid expansion with left shift, Fe overlaoed in 2015, reactive lymphocytosis and mastocyte infiltration (more close to AA).
ugnius
2017-01-14 15:09
In 2016: there is no difference in T population (CD2/CD3/CD5 > CD7+), scattered CD57+ lymphos....
tzankov
2017-01-16 07:04
Well, could be AA, but give to the hematologist an advise to activelly look for T-LGL in the PB and perfrom FACS to address this question
ugnius
2017-01-16 07:42
Thanx. All recomendations due to minor populations added.
tanvira
2017-01-30 02:11
The panorama has an appearance of hotspot of hematopoietic activities in the midst of aplastic background, which goes in favor of regenerating AA given the history.
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Last modified: 2017-01-09 15:48:22