American Journal of Hematology 41 :184-189 (1992)
Accessory Spleen in Recurrent Chronic Immune Thrombocytopenic Purpura T. Facon, M.T. Caulier, P. Fenaux, 1. Plantier, X. Marchandise, M. Ribet, J.P. Jouet, and F. Bauters Service des Maladies du Sang (T.F., M.T.C., P.F., I.P., J.P.J., F.B.), Service de Medecine Nucleaire (M.T.C., X.M.), Service de Chirurgie (M.R.), C.H.U. Lille, France
From 1969 to 1985 we discovered accessory spleens in 8 patients with chronic immune thrombocytopenic purpura (ITP) who relapsed or failed after splenectomy. Imaging of accessory spleen used a liver spleen scintigraphy with heat-treated RBC labeled with Tc-99m. Platelet kinetic studies with 51Cror "'In, including sequestration index, were performed. Five patients had accessory splenectomy. Disappearanceof bleeding symptoms was achieved in the 5 splenectomized patients but with only partial response of platelet counts. These results are discussed in the context of diagnosis and therapeutic management of accessory spleens in patients with chronic immune thrombocytopenic purpurawho relapsedor failed afler splenectomy. D 1992 Wiley-Liss, Inc. Key words: chronic immune thrombocytopenic purpura, accessory spleen, platelet kinetic study
INTRODUCTION
Splenectomy remains the most active therapeutic approach in chronic immune thrombocytopenic purpura (ITP) with response rates ranging from 60 to 90% [ 1-31. In patients who relapse or fail after splenectomy various treatments have been used, including vinca alkaloids [4-6], azathioprine [7], danazol [8] , or a-interferon (IFN-a) [9]. The development of accessory spleens may play a role in the recurrence of chronic ITP after splenectomy [10,11]. However, the diagnostic procedures in this context have varied considerably and the value of accessory splenectomy remains unclear. We reviewed here our experience in 8 patients with accessory spleen and chronic ITP relapse or failure after splenectomy and discussed the therapeutic attitude in these patients.
labeled platelets ( 1 and predominantly hepatic if ASlL was < 1. RESULTS
The characteristics of the 8 patients with chronic ITP relapse (7 patients) or failure (1 patient) after splenectomy who had an accessory spleen are shown in Table I. All these patients had a reduced lifespan of their labeled platelets. There were 7 female and 1 male patients. Re-
185
lapse after splenectomy had occurred between 9 months and 21 years and in 2 patients relapse occurred during pregnancy. In 4 patients, Howell-Jolly bodies had not appeared at any time after splenectomy. By contrast, Howell-Jolly bodies had appeared in the other 4 patients with disappearance at relapse in 1 patient. Liver spleen scintigraphy with heated Tc-99m-RBC revealed one or two accessory spleen. The accessory spleens ranged in size from 2-4 cm in largest dimension. When an accessory spleen was identified, platelet kinetic studies ("Cr in 3 patients, I 'In in 4 patients) including sequestration index were performed. Visualization of the accessory spleen by the scintigraphy and study of platelet sequestration for patient 1 are shown in Figures I and 2. Accessory splenectomy was performed in 5 patients and we chose to delay the surgical procedure in 3 patients. No mortality or morbidity was associated with accessory splenectomy . In 1 patient only, a CT scan of the abdomen was performed in order to confirm the localization of the accessory spleen but its result was negative. In the patients who underwent splenectomy , platelet counts raised from less than 50 X 109/L to 65-130 X 10'/L. Nevertheless, all patients had significant bleeding (purpura in 5 patients, genital bleeding in 2 patients, epistaxis in 2 patients) before accessory splenectomy which disappeared after surgery. Before surgery no patients had Howell-Jolly bodies (in patient 3 , Howell-Jolly bodies had disappeared at relapse after first splenectomy), but they reappeared after accessory splenectomy in all cases. In three patients, we made the choice to delay accessory splenectomy. Patient 6 had a small size accessory spleen with predominant liver sequestration and HowellJolly bodies on the peripheral blood smear. She had 4 relapses during a 12-year period that were always sensitive to azathioprine. The platelet count has been normal for 3 years without any treatment and the function of her accessory spleen is very questionable. Patient 7 is on azathioprine treatment with a platelet count of 4050 x 109/L. Despite predominantly hepatic sequestration she will probably soon be considered for accessory splenectomy. Patient 8 has a small accessory spleen with predominantly splenic sequestration but her platelet count is 50-100 x 1O'/L without any treatment. The splenectomy would only be considered in this patient in case of severe relapse. DISCUSSION
It is usually accepted that the presence of a functioning accessory spleen should be considered in all patients with chronic ITP who fail to respond or relapse following initial splenectomy . In our series, we found an accessory spleen in 8 of 65 patients (12.3%) tested between 1969 and 1985. In the vast majority of our patients, the search of accessory spleen was performed when patients re-
+
+
24
5
M
F
F
F
F
6
7
8
+ 2 (during pregnancy) 21
5 12 (during pregnancy) 0.75
4 Failure 7
Time of relapse (years)
1; ND
2; ND
1; ND
2; 4 and 2 cm 2; 4 and 2 cm
I ; 3.5 cm 1; ND 2: 4 and 2 cm
Accessory spleen (number, size)
1
1
2
2 2
4
Accessory Spleen in Recurrent Chronic Immune Thrombocytopenic Purpura T. Facon, M.T. Caulier, P. Fenaux, 1. Plantier, X. Marchandise, M. Ribet, J.P. Jouet, and F. Bauters Service des Maladies du Sang (T.F., M.T.C., P.F., I.P., J.P.J., F.B.), Service de Medecine Nucleaire (M.T.C., X.M.), Service de Chirurgie (M.R.), C.H.U. Lille, France
From 1969 to 1985 we discovered accessory spleens in 8 patients with chronic immune thrombocytopenic purpura (ITP) who relapsed or failed after splenectomy. Imaging of accessory spleen used a liver spleen scintigraphy with heat-treated RBC labeled with Tc-99m. Platelet kinetic studies with 51Cror "'In, including sequestration index, were performed. Five patients had accessory splenectomy. Disappearanceof bleeding symptoms was achieved in the 5 splenectomized patients but with only partial response of platelet counts. These results are discussed in the context of diagnosis and therapeutic management of accessory spleens in patients with chronic immune thrombocytopenic purpurawho relapsedor failed afler splenectomy. D 1992 Wiley-Liss, Inc. Key words: chronic immune thrombocytopenic purpura, accessory spleen, platelet kinetic study
INTRODUCTION
Splenectomy remains the most active therapeutic approach in chronic immune thrombocytopenic purpura (ITP) with response rates ranging from 60 to 90% [ 1-31. In patients who relapse or fail after splenectomy various treatments have been used, including vinca alkaloids [4-6], azathioprine [7], danazol [8] , or a-interferon (IFN-a) [9]. The development of accessory spleens may play a role in the recurrence of chronic ITP after splenectomy [10,11]. However, the diagnostic procedures in this context have varied considerably and the value of accessory splenectomy remains unclear. We reviewed here our experience in 8 patients with accessory spleen and chronic ITP relapse or failure after splenectomy and discussed the therapeutic attitude in these patients.
labeled platelets ( 1 and predominantly hepatic if ASlL was < 1. RESULTS
The characteristics of the 8 patients with chronic ITP relapse (7 patients) or failure (1 patient) after splenectomy who had an accessory spleen are shown in Table I. All these patients had a reduced lifespan of their labeled platelets. There were 7 female and 1 male patients. Re-
185
lapse after splenectomy had occurred between 9 months and 21 years and in 2 patients relapse occurred during pregnancy. In 4 patients, Howell-Jolly bodies had not appeared at any time after splenectomy. By contrast, Howell-Jolly bodies had appeared in the other 4 patients with disappearance at relapse in 1 patient. Liver spleen scintigraphy with heated Tc-99m-RBC revealed one or two accessory spleen. The accessory spleens ranged in size from 2-4 cm in largest dimension. When an accessory spleen was identified, platelet kinetic studies ("Cr in 3 patients, I 'In in 4 patients) including sequestration index were performed. Visualization of the accessory spleen by the scintigraphy and study of platelet sequestration for patient 1 are shown in Figures I and 2. Accessory splenectomy was performed in 5 patients and we chose to delay the surgical procedure in 3 patients. No mortality or morbidity was associated with accessory splenectomy . In 1 patient only, a CT scan of the abdomen was performed in order to confirm the localization of the accessory spleen but its result was negative. In the patients who underwent splenectomy , platelet counts raised from less than 50 X 109/L to 65-130 X 10'/L. Nevertheless, all patients had significant bleeding (purpura in 5 patients, genital bleeding in 2 patients, epistaxis in 2 patients) before accessory splenectomy which disappeared after surgery. Before surgery no patients had Howell-Jolly bodies (in patient 3 , Howell-Jolly bodies had disappeared at relapse after first splenectomy), but they reappeared after accessory splenectomy in all cases. In three patients, we made the choice to delay accessory splenectomy. Patient 6 had a small size accessory spleen with predominant liver sequestration and HowellJolly bodies on the peripheral blood smear. She had 4 relapses during a 12-year period that were always sensitive to azathioprine. The platelet count has been normal for 3 years without any treatment and the function of her accessory spleen is very questionable. Patient 7 is on azathioprine treatment with a platelet count of 4050 x 109/L. Despite predominantly hepatic sequestration she will probably soon be considered for accessory splenectomy. Patient 8 has a small accessory spleen with predominantly splenic sequestration but her platelet count is 50-100 x 1O'/L without any treatment. The splenectomy would only be considered in this patient in case of severe relapse. DISCUSSION
It is usually accepted that the presence of a functioning accessory spleen should be considered in all patients with chronic ITP who fail to respond or relapse following initial splenectomy . In our series, we found an accessory spleen in 8 of 65 patients (12.3%) tested between 1969 and 1985. In the vast majority of our patients, the search of accessory spleen was performed when patients re-
+
+
24
5
M
F
F
F
F
6
7
8
+ 2 (during pregnancy) 21
5 12 (during pregnancy) 0.75
4 Failure 7
Time of relapse (years)
1; ND
2; ND
1; ND
2; 4 and 2 cm 2; 4 and 2 cm
I ; 3.5 cm 1; ND 2: 4 and 2 cm
Accessory spleen (number, size)
1
1
2
2 2
4
