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BM biopsy (1673)
BM biopsynew
Subtitle: HEMATO
Type:
Biopsy
Sender:
ugnius
2006-03-22 08:54
INCTR - EBMWG Hematopathology Online
BM biopsy (clot) to 74yrs male was performed. CLIN. INFO: Philadelphia chromosome (-), splenomegaly, pancytopenia, Hb 96 g/, Platelets 21, leukocytes 8,0 without left shift, the lack of normoblasts. BM aspirate with expanded granulopoesis with left shift, megakaryocytosis, single micromegakaryocytes. There are any skin rush.  
CLIN DGN: IDIOPATIC MYELOFIBROSIS?  
HISTO: on photos bellow.  
IH: spindle, "epithelioid" clear cells with ovoid nuclei are CD117+++, CD68+ (I've not mast cell tryptase).  
PROPOSED DIAGNOSIS: Chronic myloproliferative disease (cellular myelofibrosis?) with systemic mastocytosis vs systemic mastocytosis only.  
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hurwitz
2006-03-22 13:41
Good timing, I was just about to ask for CD117 and CD68 stains, and here they came. As you said there is no doubt that this is systemic mastocytosis. The perivascular CD117 and CD68+ cells a rather atypical. Some authors regards cellular atypia in SM as a criterion for aggressiveness.  
I wonder whether your question about an associated MPD can be answered unequivocally. The marrow is defintely hypercellular, with marked myeloid hyperplasia and left shift as you correctly stated. Megakaryocytes morphology is unremarkable. There is one small megakaryocyte (I would not call this cell micromegakaryo-  
cyte). Erthropoiesis is present but markedly decreased. Pb values do notpoint to an MPD either. My feeling is that the hypercellularity is due to the heavy MCD infiltrate with assocoated myeloid hyperplasia, eosinophilia and lymphocytosis. However I cannot say this with certainity. Since CMML many be often associated with MC, it would be interesting to know if there is a monocytosis in the PB.
ugnius
2006-03-22 15:06
Nice to hear form you dr.N.Hurwitz. Today we with Kurt translate iPath to lithuanian:)  
About this case: Giemsa (-)(except some typical mastocytes). I will add some photos about fibrosis and mega's.
ugnius
2006-03-22 15:16
a lot of appologies for defect meg's picture.
hurwitz
2006-03-22 16:06
Congratulations to the Lithuanian version of iPath.  
Thanks for the additional Giemsa stained images. Indeed there are no mastcell granules to be seen, but loss of granulation can be a feature of atypical mast cells. There is marked fibrosis,but this is still no proof for an associated MPD. The megas, you added, image 1 in are hypolobated, which is a pathologic feature, but again not enough to allow conclusions.  
I forgot to mention that I added a review on MCD as PDF file
ugnius
2006-03-22 18:10
Thank you for pdf. For sure in this case we have definite mastocytosis with III-IV fibrosis and some myeloproliferation, maybe reactive. Thank you.
ffrenchma
2006-03-28 15:08
-BM biopsy shows a mastocytosis with criteria of systemic mastocytosis.  
-Hematopoiesis persists while an anemia and a profound thrombopenia were observed.  
Megakaryocytes appear to be small with a round nucleus suggesting a dysmegakaryocytopoiesis. We cannot clearly see erythroid precursors.  
-A diagnosis of MDS associated with a systemic mastocytosis must be discussed and may be confirmed by bone marrow aspirate examination. Fibrosis may be related to both mastocytosis and MDS.
hurwitz
2006-03-28 17:18
Thanks Martine for your excellent comment. I agree that an MDS associated with the mastocytosis has to be considered. My impression is also that myelopoiesis is left shifted. I would suggest to do a CD34 stain as well
aorazi
2006-04-06 14:02
Interesting case. A diagnosis of mastocytosis is established. CIMF is ruled out morphologically.  
 
Associated hematopoietic malignancy. I totally agree with Martine and Nina. A diagnosis of MDS or MDS/MPD (how many monocytes?) associated with systemic mastocytosis may be entertained. However, to diagnose MDS or MDS/MPD in this case, bone marrow aspirate and PB smear need to be carefully reviewed. . Bone marrow biopsy changes seen in your pictures are per se non diagnostic. Please use standard units (? e.g. leucocytes 8,0 meaning 8,000 /dl or else). Hypersplenism associated with mastocytosis may play a role in the "cytopenias" seen here. CD34 is a good idea to highlight blasts. Iron stain need also to be considered, but, most important -- you just report Ph' negativity --- what about the rest of the karyotype?
ugnius
2006-04-07 19:42
My dears Prof.Nina, dr.Martine, dr.Atilio, thank you for guidance. The case is sent from Klaipeda hospital of Marines (Klaipeda region usually attacks us with VERY and VERY good cases in hematology and overall). So I will check clin details 'cause I was not involved directly. I will try to join clinicians from there, 'cause I cannot answer to your complicated questions about "virtual" (for me) patient. With the best wishes.  
SPECIAL QUEST's for dr.Atilio: 1. I've heard so much scepticism regarding Fe stains in BM biopsy (by the way from US collegues). Do you believe in it's value?  
2. What do you recommend for MDS diagnosis (descriptive for sure) formulation on threpines, if we are still working in German model (with L/N and BM only). And aspirate/blood films are in a distance from us. Thanx.
hurwitz
2006-04-08 15:33
Dear Ugnius, in general I do not believe in separating biopsies and aspirates, both are basic intergral parts of bone marrow examination, and are complimentary methods regarding the information you can obtain. This means we need both. In the US both are performed by the hematopathologist, in Europe we have the schisma between hematopogists and hematopathologists, but as long as both communicate well this shisma can be bridged.In the present case as Martine said, there are abnormal megakaryocytes, and myelopoiesis appears left shifted, however, we need additional information from the aspirate to clarify the question MDS/ MPD/MDS: are there signs of dysplastic myelopiesis (which you cannot see on the biopsy), or dysplastic erythropoiesis including rinsideroblasts? This is the reason why you need an ironstain on the aspirate. I agree iron stain on the biopsy is of limited value. I also agree with Attilio that we need to know if there is monocytosis and how long does it persist.
ugnius
2006-04-09 14:51
Dear collegues: Fe stain reveals minimal deposition in BM. CD34+ interstitial "blast" population is a little bit expanded, but still <5%. We are waiting add clin data. I've apend "MDS/CMPD possibility" together with mastocytosis dgn. in report. Thank you for assistance.
aorazi
2006-04-10 12:47
Iron staining issues:  
In my reply I meant on the aspirate. If the aspirate is handled by your clinical hematologist colleagues, then get them to do it! Remind them that the test is mandated by both FAB and WHO.  
 
Iron & BM biopsy. US pathologists are skeptical simply because they use nitric acid to decalcify, a treatment which "decrease" ficticiously the amount of stainable iron present in the histology section. If you use EDTA, than it is not too bad. I still rely on the air dried smears though. One way around the problem -- you can try the clot sections (which is not decalcified). Best tegards to all.
ugnius
2006-04-10 13:01
Thank you dr.A.Orazi. My Fe stain is esthetic enough using EDTA. In this case information somes too slowly from Klaipeda hospital managing the patient. So I have nothing to add till now.  
Thank you for suporting.
hurwitz
2006-04-10 13:40
[comment sent by email]
Question to Attilio, how useful are clot sections for the
 
demonstration of ringsideroblasts?
 
 
aorazi
2006-04-13 12:38
Nina:  
 
Q. ringed sideroblasts and BM clot sections. Clots work if particulated enough and cut at 3um max. Still the aspirate is more sensitive.
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Last modified: 2006-03-22 08:54:46