A 76 years old man, followed since 1999 because of homogeneous echographic splenomegaly. Hb levels were normal (13.2 g/dl) with 50000 platelets/ml and 3900 leucocytes (75% PMN). A bone marrow examination disclosed hypercellularity. As ITP could not be ruled out, danazol 400 mg/day was started and maintained during 3 months without any increase of platelet levels. His platelet counts remained between 45000 and 80000 during follow-up. In February 2006, a heterogeneous spleen was seen during an abdominal ultrasound and confirmed with a CT scan. A new bone marrow examination was slightly hyper cellular for his age and no abnormal lymphoid populations were found by FACS or immunohistochemistry. Splenectomy was performed in June 2006. Post op platelet levels increased to 449.000. Spleen was slightly enlarged, with reddish surface.

Vijnovich-Baron 2006-07-21 06:06	Microscopy: There was a 10 cm area with no white pulp, and many macropahges, blood dilates spaces and marked extramedullary hemopoiesis. Images shown H&E, FVIII, CD68, CD34 and CD31. CD8 ws negative in the blood filled spaces wall.
anpo 2006-07-23 13:40	Isolated peliosis of spleen is very rare - usually there are some changes also in the liver. Was liver CT or ultrasound performed? As far as I know extensive extramedullary hematopoiesis is not the main characteristics of peliosis.   Also it would be interesting to see the bone marrow biopsy if available to rule out some MDS/MPD. Was there any fibrosis? However, extramedullary hematopoiesis can present as a mass in a spleen in patients without hematopoietic disorder (Acta Cytol. 2002 Nov-Dec;46(6):1138-42.).
hurwitz 2006-07-24 23:38	I am quite sure that extramedullary hematopoiesis is not a feature of splenic peliosis, but dilated splenic sinusoids are usually seen in EMH. I agree with Anja that EMH in the spleen can be seen without any hematologic disorder, but in these cases I would not expect it to be as massive as in the submitted case. Therefore I think Anjas suggestion is very important, we have to see the bone marrow biopsy, if possible both, the recent and the previous one. Anahi mentiones hypercellularity in both biopsies. Bone marrow changes in cellular phase of MF can be very subtle and easy to overlook.
Vijnovich-Baron 2006-07-25 15:52	Thanks for the comments.   Liver ultrasound was normal     I will take some bone marrow digital pictures.     Patient had Danazol that is androgen derivated, and might be related to peliosis.     I didn't find any relationship of EMH and peliosis as is related to hammartoma.
hurwitz 2006-07-25 22:17	It would be interesting to see an incubation with CD8, since endothelial cells of normal splenic sinusoids as well as in peliosis do express CD8, in contrast to endothelial cells in hemagiomas which are CD8-.
Went 2006-07-26 14:41	Peliosis lienalis is listed as a possible side effect of Danazol. A combination of factors may be responsible for the seen picture (EMB & side effect?). A macroscopic picture could help to resolve the problem regarding the differential diagnosis of Peliosis. If the cut surface was homogenous reddish as suggested, this would strongly argue against Peliosis, in which blood-filled cavities would be expected.
aorazi 2006-07-27 18:37	I agree with Nina's and Anja's reservations concerning the vascular nature of the lesions. The degree of EMH and the somewhat atypical cytologic characteristics of the megakaryocytes and their arrangements into tight clusters in a thrombocytopenic patient with some splenomegaly (spleen weight?) strongly suggest CIMF or some related CMPD. The marrow is reported as hypercelluar which is unusal in chronic ITP. No mention of the megakaryocyte morphology and the reticulin content as seen in the BM biopsy. In conclusion I favor vascular ectasia with some fibrosis in association with sigificant EMH suggestive of a CMPD.
hurwitz 2006-07-27 21:05	I forgot to mention in my comment that some megas do appear atypical, see drawing.
Vijnovich-Baron 2006-07-29 02:36	I added bone marrow images.   As I said slighty hypercellular for 76 years old. Cellularity was irregularly distributed.     Doesn't look as Myeloproliferative disorder to me.
hurwitz 2006-07-29 15:09	Dear Anahi, I agree with you at the first glance this marrow does not look myeloproliferative at all. Still, could you please be so kind and provide us some high power images to see morphologic details of all cell lines.
dirnhofer 2006-08-05 20:38	as to me, the marrow looks hypercellular but otherwise normal (slight eosinophilia); morphologic criteria for a mpd are not fulfilled.   what was the size and weight of the spleen? could hypersplenism explain the thrombopenia?
hurwitz 2006-08-15 20:42	Dear Anahi, we stil do not have any satisfying ansver to this case. In such a difficult case we have to try to collect all available information. You gave us images of one bone marrow biopsy, which one is it? from 1999 or 2006. Could you please sent us images from the missing biopsy? In addition Philip Went's suggestion to get a macroscopic image of the spleen, or if not available a macroscopic description including the weight might be helpful.We need to know if the cutsurface was homogenous or nodular. I also marked an area rich in megas on image dscn 2646, from which I would appreciate a high power image to see their morphology.   Sorry I must have overlooked that you said that CD8 was negative in the vessel walls. Could you see CD8+ blood filled spaces in other areas?
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