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BONE MARROW FAILURE: AA LIKE (817290) » 2015 CD117.jpg
Filename: 2015 CD117.jpg
[BONE MARROW FAILURE: AA LIKE]
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.  
 
 
Sender: ugnius
2017-01-14 15:04
INCTR - EBMWG Hematopathology Online

Last modified: 2017-01-14 15:04:26