Thrombocytopenia in sarcoidosis.
BACKGROUND
Haematological manifestations in sarcoidosis are uncommon. The prevalence of thrombocytopenia in sarcoidosis is not well assessed.


OBJECTIVE
To describe the main characteristics and outcome of sarcoidosis associated with thrombocytopenia.


METHODS
We described 2 personal cases and a complete record of all reports of thrombocytopenia in sarcoidosis was persuaded through a medline multi language computer search from 1972 until now.


RESULTS



METHODS
In the first observation the clinical course was similar to immune thrombocytopenic purpura. Steroids were efficient. In the second, we have reported the first used of Rituximab in thrombocytopenia in sarcoidosis with a partial success.


METHODS
We identified three main physiopathological mechanisms among the 31 cases collected. Hypersplenism or splenomegaly was found in ten cases, granulomas in bone marrow were found in only four. Auto-immune thrombocytopenic purpura was suspected in the other cases. 23 patients had been treated with steroids, which proved effective in 21 cases (in association with intravenous immunoglobulin(IV-ig) or anti-D. Among the five cases for which steroids were non efficient, subsequent splenectomy allowed normalization of platelets count. Splenectomy was performed in seven cases, as a first intention treatment for five patients, and successful in four. One patient died of massive haemorrhage during the surgery. Among the 5 patients treated with IV-Ig, 4 had a complete response.


CONCLUSIONS
Different physiopathological mechanisms are responsible of thrombocytopenia in sarcoidosis. Granulomas in bone marrow or hypersplenism may be involved. Immune thrombocytopenic purpura must be suspected in all other cases. Steroids remain the most effective treatment, and must be proposed in first intention.
Mahévas
Matthieu
M
Department of Internal Medicine, CHU Nord, Amiens Cedex 1, France. morganafara@hotmail.com
Le Page
Laurence
L
Salle
Valery
V
Lescure
Francois-Xavier
FX
Smail
Amar
A
Cevallos
Ramiro
R
Garidi
Redha
R
Marolleau
Jean-Pierre
JP
Duhaut
Pierre
P
Ducroix
Jean-Pierre
JP
D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D000069283:Rituximab; D017565:Sarcoidosis, Pulmonary / Q000150:complications*; D013256:Steroids / Q000627:therapeutic use; D013921:Thrombocytopenia / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D013256:Steroids; D000069283:Rituximab