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Spleen, DD reactive changes versus infiltrates by hematologic neoplasia (3673)
Spleen, DD reactive changes versus infiltrates by hematologic neoplasiaclosed
Subtitle: b07-23946
Type:
HEMATO
Sender:
ugnius
2007-09-06 13:58
INCTR - EBMWG Hematopathology Online
53 yrs old man underwent splenectomy due to hemoperitoneum and intraabdominal bleeding clinics.  
MACRO: Spleen 7,5x6x2,5cm with tears up to 3,5cm inthe capsule.  
HISTO: The infiltration of CD68+ histyoid cells with irregular deformed nuclei, eosinohilic PAS+ cytoplasm and immature CD34- MPO+ CD34- CD1a- S100- and mature myoeloid cell infiltration in the sinuses expanding the red pulp. There are some micoabscesses with eosinophils.  
FULL IH: Histyoid cells: CD68(+++) 100%, S100(++)20%, CD1a/MPO(-), CD4(+/++) 90%, Ig kappa/lambda (+/++)70%, Bcl2(-), Hb(+)40%.  
Immature myeloid population: MPO+ NASDE+CD34-CD117-. Ki67 in red pulp 30%. Follicles and sparse agregates: CD20+. Peritrabecular and interstitial lymphos: CD3+/Bcl2+. CD4/CD8 ~ 1/1. CD30+ single cells. Plasmacytes (partly- CD138+/Bcl2+) polytypic: Ig kappa/Ig lambda+. Single CD117+ mastocytes. Single CD61+ megakaryocytes. Sinusoids: CD8+.  
CLINICS: The patient underwent polytrauma and died son after laparotomy.  
 
PROPOSAL: Extramedulary hematopoesis due to myeloproliferative disease (CML? oth.?) with possible erythrophagocytosis? OTHER?  
 
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Mueller-Hermelink
2007-09-15 17:10
In my view ,this is no neoplastic disease but rather infectious and reactive spleen swelling. These abszesses are probably due to a bacteriaql disease .
hurwitz
2007-09-16 19:04
I prefer not to comment on the case without additional information: size and weight of the spleen? reason for rupture ? adequate trauma?  
Clinical history: evidence of preceeding hematologic disorder?  
PBC? Was a bone marrow examination performed: aspirate or BMB,or flowcytometry? If yes please let us know the results.  
ugnius
2007-09-17 08:40
Some data added. The patient died soon after procedure (polytrauma). I've reguested clin data from the peripheral hospital but do not believe in success.  
 
Mainly my question is about "strange" macrophages with irregular nuclei.  
Thank you.
diane.c.farhi
2007-09-19 18:26
Hello to all; I am new to this group and appreciate the opportunity to see these cases. In my view, this is a malignant infiltrate; the interfollicular location of the infiltrate is typical of leukemia, myeloproliferative disorders (MPD), and myelodysplastic syndromes. I believe that it is likely an infiltrate of acute myeloid leukemia, possibly with eosinophilic and monocytic differentiation (it's hard for me to tell on these photos). MPD typically shows splenomegaly and megakaryocytic proliferation, which are minimal to absent here. The diagnosis of leukemia is supported by the location of the infiltrate, numerous mitoses, eosinophilic myelocytes, positivity for MPO, CD4, CD68 (monocytic differentiation), and hemoglobin (erythroid differentiation). Thank you again for allowing me to offer my opinion.
hurwitz
2007-09-30 17:39
I agree with Diane's comment, this is splenic involvement by a malignant hematologic disorder, a reliable more exact characterization of the process cannot be made on this material.  
However, I will allow myself to present some speculations: There are large clusters of MPO+ immature myeloid cells, and numerous CD4/CD68+ cells, probably of monocytic origin. The prominent eosinophilia, with immature forms accompanying the infiltrates could point to an extramedullary manifestation of an AMML eo. I wonder if the eosinophilic granula show abnormal coarse forms on oil immersion, as seen in AMML eo? An incubation with CD117, might give more information on the amount of immature myeloid cells.  
 
SergeyN
2007-10-01 09:41
The presence of multiple HGB+ nucleated cells should be taken into account somehow.  
 
Two questions:  
 
- how do eosinophils topographically relate to MPO+ foci? Are they an integral part?  
 
- what is the topography of HGB+ cells? Are they diffuse throughout the red pulp or go together with myeloid infiltration?  
 
And I, too, think that CD117 should be checked. It is a very unusual myeloproliferation with compact focal and intrasinusoidal MPO+CD34- infiltrates, moderate monocytic component, multiple red cells and only occasional mega, small at that. CMML should not be completely ruled out.  
 
 
 
ugnius
2007-10-01 09:52
Please find full IH description above. I'm sorry, but any aditional data arrive maybe due to fatality of the case.
hurwitz
2007-10-01 16:22
I absolutely agree with you Sergey, CMML has to be considered as well. The patient mmight have suffered from CMML, which was clinically not apparent. Probably we will never be able to solve the case on a more solid base apart from speculations.  
Ugnius,could you please submit some high pover images of HB+ cells, is there any erythrophagocitosis? I would also appreciate some high power images of the eosinophils, some of them do not look quite normal to me.
anpo
2007-10-14 00:40
I just tonight had time to review this interseting case. I must say that I agree with Prof. Muller-hermalink and would favor the suspicion of rupture due to infection. As far as I understand the spleen was of normal size/weight and the pattern of infiltration by CD68+ cells is not clearly characteristic for MPD.
ugnius
2007-10-15 08:54
Thank you for a comments. Please find some pictures requested by Prof.Nina. In this case the strange form of nuclei of histyocytes was slightly unusual for me.
anpo
2007-10-20 23:31
The pattern of MPO is unusual but not entirely characteristic of MPD. I think that we will not solve this case without more clinical data - Blood status? Signs of infection? Trauma?
hurwitz
2007-11-05 21:34
Because of lack of clinical data we can not reach a diagnostic conclusion. The DD will remain betweeen changes reactive to trauma and/or infection (K.Müller-Hermelink, A.Porwit) and infiltration by a hematologic neoplasia, CMML? (Farhi, Nikulshin, Hurwitz). Again a case demonstrating the importance of clinico pathologic correlations in hematopathologic diagnosis.
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Last modified: 2007-09-06 13:58:32