Isolated Diffuse Hemangiomatosis of the Spleen with Disseminated Intravascular Coagulation: Successful Treatment with Embolization and Splenectomy
A
1-year-old boy was hospitalized because of a 2-month history of abdominal distention. Physical examination revealed splenomegaly and purpura throughout his body. Laboratory results confirmed the presence of severe disseminated intravascular coagulopathy (DIC). Therefore, the patient was treated with daily transfusions of platelets and fresh frozen plasma. Computer tomography revealed an enlarged spleen, with diffuse, net-like enhanced lesions throughout the spleen (Figure 1). He was diagnosed with splenic hemangioma and treated with prednisolone, propranolol, and vincristine. However, the patient’s condition worsened, and he was transferred to our hospital. At admission, he experienced high-output cardiac failure, attributable to transfusion-related overload and pooling blood in the enlarged spleen. Because he was unable to undergo open splenectomy due to his status, we performed partial embolization. We occluded the splenic artery at the splenic hilum, thereby preserving the superior polar artery, the pancreatic branches, and the left gastroepiploic artery (Figure 2; available at www.jpeds.com). After the embolization, the
spleen decreased in size, and the frequency of the transfusions was subsequently reduced. The patient underwent successful splenectomy 1 week after embolization; histology indicated that the small vessels were diffusely distributed throughout the spleen, with endothelial cells that were diffusely positive for CD34 and factor VIII, and focally positive for CD31 and CD8. Based on this pathologic finding, the patient was diagnosed with splenic hemangiomatosis, and was discharged 24 days after the splenectomy, with no evidence of DIC or heart failure. Isolated splenic hemangiomatosis is a rare disorder, which can occur at any age and is characterized by splenomegaly and vascular channels that penetrate the entire spleen.1-3 Although thrombocytopenia and coagulation disturbance are observed in approximately one-half of these cases, the reported standard therapy is splenectomy. In conclusion, partial embolization and subsequent splenectomy may be an appropriate treatment for splenic hemangiomatosis with DIC. n Yoshiko Nakano, MD Hiroyuki Fujisaki, MD, PhD Department of Pediatric Hematology and Oncology
Tomoya Ishiguro, MD, PhD Department of Neurosurgery
Takao Manabe, MD, PhD Department of Radiology
Takeshi Inoue, MD, PhD Department of Pathology Osaka City General Hospital
Young-Dong Park, MD, PhD Department of Pediatrics Osaka Red Cross Hospital
Tetsuro Nakamura, MD, PhD Department of Pediatric Surgery
Junichi Hara, MD, PhD Figure 1. Enhanced computed tomography scanning reveals an enlarged spleen occupying the abdominal cavity.
Department of Pediatric Hematology and Oncology Osaka City General Hospital Osaka, Japan
References available at www.jpeds.com J Pediatr 2015;166:1093. 0022-3476/$ - see front matter. Copyright ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.12.033
1093
THE JOURNAL OF PEDIATRICS
www.jpeds.com
References 1. Dufau JP, le Tourneau A, Audouin J, Delmer A, Diebold J. Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome. Histopathology 1999;35:337-44.
Vol. 166, No. 4 2. D’Antonio A, Boscaino A, De Dominicis G, Nappi O. Splenic hemangiomatosis. A report of two cases and review of literature. Adv Clin Path 2002;6:119-24. 3. Steininger H, Pfofe D, Marquardt L, Sauer H, Markwat R. Isolated diffuse hemangiomatosis of the spleen: case report and review of literature. Pathol Res Pract 2004;200:479-85.
Figure 2. Digital subtraction angiography reveals A, the enlarged spleen before the occlusion and B, after the occlusion of the inferior two-thirds of the spleen. 1093.e1
Nakano et al
A
1-year-old boy was hospitalized because of a 2-month history of abdominal distention. Physical examination revealed splenomegaly and purpura throughout his body. Laboratory results confirmed the presence of severe disseminated intravascular coagulopathy (DIC). Therefore, the patient was treated with daily transfusions of platelets and fresh frozen plasma. Computer tomography revealed an enlarged spleen, with diffuse, net-like enhanced lesions throughout the spleen (Figure 1). He was diagnosed with splenic hemangioma and treated with prednisolone, propranolol, and vincristine. However, the patient’s condition worsened, and he was transferred to our hospital. At admission, he experienced high-output cardiac failure, attributable to transfusion-related overload and pooling blood in the enlarged spleen. Because he was unable to undergo open splenectomy due to his status, we performed partial embolization. We occluded the splenic artery at the splenic hilum, thereby preserving the superior polar artery, the pancreatic branches, and the left gastroepiploic artery (Figure 2; available at www.jpeds.com). After the embolization, the
spleen decreased in size, and the frequency of the transfusions was subsequently reduced. The patient underwent successful splenectomy 1 week after embolization; histology indicated that the small vessels were diffusely distributed throughout the spleen, with endothelial cells that were diffusely positive for CD34 and factor VIII, and focally positive for CD31 and CD8. Based on this pathologic finding, the patient was diagnosed with splenic hemangiomatosis, and was discharged 24 days after the splenectomy, with no evidence of DIC or heart failure. Isolated splenic hemangiomatosis is a rare disorder, which can occur at any age and is characterized by splenomegaly and vascular channels that penetrate the entire spleen.1-3 Although thrombocytopenia and coagulation disturbance are observed in approximately one-half of these cases, the reported standard therapy is splenectomy. In conclusion, partial embolization and subsequent splenectomy may be an appropriate treatment for splenic hemangiomatosis with DIC. n Yoshiko Nakano, MD Hiroyuki Fujisaki, MD, PhD Department of Pediatric Hematology and Oncology
Tomoya Ishiguro, MD, PhD Department of Neurosurgery
Takao Manabe, MD, PhD Department of Radiology
Takeshi Inoue, MD, PhD Department of Pathology Osaka City General Hospital
Young-Dong Park, MD, PhD Department of Pediatrics Osaka Red Cross Hospital
Tetsuro Nakamura, MD, PhD Department of Pediatric Surgery
Junichi Hara, MD, PhD Figure 1. Enhanced computed tomography scanning reveals an enlarged spleen occupying the abdominal cavity.
Department of Pediatric Hematology and Oncology Osaka City General Hospital Osaka, Japan
References available at www.jpeds.com J Pediatr 2015;166:1093. 0022-3476/$ - see front matter. Copyright ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.12.033
1093
THE JOURNAL OF PEDIATRICS
www.jpeds.com
References 1. Dufau JP, le Tourneau A, Audouin J, Delmer A, Diebold J. Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome. Histopathology 1999;35:337-44.
Vol. 166, No. 4 2. D’Antonio A, Boscaino A, De Dominicis G, Nappi O. Splenic hemangiomatosis. A report of two cases and review of literature. Adv Clin Path 2002;6:119-24. 3. Steininger H, Pfofe D, Marquardt L, Sauer H, Markwat R. Isolated diffuse hemangiomatosis of the spleen: case report and review of literature. Pathol Res Pract 2004;200:479-85.
Figure 2. Digital subtraction angiography reveals A, the enlarged spleen before the occlusion and B, after the occlusion of the inferior two-thirds of the spleen. 1093.e1
Nakano et al
