Partial splenic embolization in a child with Gaucher disease, massive splenomegaly and severe thrombocytopenia

被引:5
|
作者
Pena, Andres H. [1 ]
Kaplan, Paige [2 ]
Ganesh, Jaya [2 ]
Clevac, Egor [1 ]
Cahill, Anne Marie [1 ]
机构
[1] Childrens Hosp Philadelphia, Dept Radiol, Philadelphia, PA 19104 USA
[2] Childrens Hosp Philadelphia, Div Metab Dis, Philadelphia, PA 19104 USA
关键词
Gaucher disease; Partial splenic embolization; Polyvinyl alcohol particles; Splenomegaly; Child; HYPERSPLENISM; HYPERTENSION; THERAPY;
D O I
10.1007/s00247-009-1303-9
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
A 13-month-old boy with Gaucher disease presented with severe thrombocytopenia, anemia and massive splenomegaly. In addition he had significant respiratory compromise caused by abdominal compartment syndrome, requiring mechanical ventilation. Because of the degree of respiratory compromise and his existing bone marrow suppression, splenic artery embolization was chosen as an alternative to splenectomy. Splenic artery embolization was performed using 355-500-A mu m polyvinyl alcohol particles, with 70% ablation achieved. Within 24 h of the procedure the platelet count had risen to greater than 70,000/mm(3) and to more than 170,000/mm(3) on postoperative day 4. At the 8-month follow-up the splenic size had decreased from 18 cm to 8 cm, with a platelet count of 578,000/mm(3). Partial splenic embolization provides a minimally invasive alternative to splenectomy in patients with Gaucher disease with massive splenomegaly and bone marrow suppression.
引用
收藏
页码:1006 / 1009
页数:4
相关论文
共 50 条
  • [41] A Multicenter Observational Study For Early Diagnosis Of Gaucher Disease In Patients With Splenomegaly and/Or Thrombocytopenia
    Motta, Irene
    Filocamo, Mirella
    Stroppiano, Marina
    Poggiali, Erika
    Dragani, Alfredo
    Domenica, Maria
    Cappellini
    BLOOD, 2013, 122 (21)
  • [42] A multicentre observational study for early diagnosis of Gaucher disease in patients with Splenomegaly and/or Thrombocytopenia
    Motta, Irene
    Filocamo, Mirella
    Poggiali, Erika
    Stroppiano, Marina
    Dragani, Alfredo
    Consonni, Dario
    Barcellini, Wilma
    Gaidano, Gianluca
    Facchini, Luca
    Specchia, Giorgina
    Cappellini, Maria Domenica
    EUROPEAN JOURNAL OF HAEMATOLOGY, 2016, 96 (04) : 352 - 359
  • [43] Prevalence of Gaucher Disease in Patients of Unknown Cause of Splenomegaly and/or Thrombocytopenia in Saudi Arabia
    Elbagoury, Marwan
    Ismail, Abdulelah
    Qadi
    Hejazi, Ayman
    Alabbas, Fahad
    El Yamany, Ghaleb
    Al Saeed, Hussain H.
    Kashari, Ohoud F.
    BLOOD, 2020, 136
  • [44] A MULTICENTER OBSERVATIONAL STUDY FOR EARLY DIAGNOSIS OF GAUCHER DISEASE IN PATIENTS WITH SPLENOMEGALY AND/OR THROMBOCYTOPENIA
    Motta, I.
    Stroppiano, M.
    Barcellini, W.
    Poggiali, E.
    Dragani, A.
    Gaidano, G.
    Merli, F.
    Cappellini, M.
    HAEMATOLOGICA, 2013, 98 : 427 - 428
  • [45] Partial splenic embolization for cancer patients with thrombocytopenia requiring systemic chemotherapy
    Kauffman, Christopher R.
    Mahvash, Armeen
    Kopetz, Scott
    Wolff, Robert A.
    Ensor, Joe
    Wallace, Michael J.
    CANCER, 2008, 112 (10) : 2283 - 2288
  • [46] Usefulness of Preoperative Partial Splenic Embolization in Hepatocellular Carcinoma and Hypersplenic Thrombocytopenia
    Yoshidome, Hiroyuki
    Kimura, Fumio
    Shimizu, Hiroaki
    Ohtsuka, Masayuki
    Kato, Atsushi
    Yoshitomi, Hideyuki
    Furukawa, Katsunori
    Takeuchi, Dan
    Takayashiki, Tsukasa
    Suda, Kosuke
    Takano, Shigetsugu
    Miyazaki, Masaru
    HEPATO-GASTROENTEROLOGY, 2011, 58 (112) : 2062 - 2066
  • [47] Analysis of predictors after partial splenic embolization for thrombocytopenia with liver cirrhosis
    Ishikawa, Toru
    Ohashi, Kazuki
    Kodama, Erina
    Kobayashi, Takamasa
    Azumi, Motoi
    Nozawa, Yujiro
    Iwanaga, Akito
    Sano, Tomoe
    Honma, Terasu
    MEDICINE, 2022, 101 (40) : E30985
  • [48] Impact of partial splenic embolization on the complications and survival in patients with cirrhotic thrombocytopenia
    Zuchini, R.
    Chen, C. Y.
    Liu, Y. S.
    Kuo, T. N.
    Cheng, P. N.
    Kao, A. W.
    Chuang, C. H.
    Wu, I. C.
    Chang, W. L.
    Chiu, Y. C.
    Chang, T. T.
    Sheu, B. S.
    Lin, X. Z.
    JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, 2010, 25 : A92 - A92
  • [49] Splenic embolization to continue chemotherapy in a child with hypersplenism-related thrombocytopenia
    Rainusso, Nino
    Gruner, Stephanie E.
    Hernandez, J. Alberto
    PEDIATRIC BLOOD & CANCER, 2021, 68 (02)
  • [50] Successful treatment of severe thrombocytopenia in a cirrhotic patient with portal hypertension using partial splenic artery embolization.
    Dahhan, W
    Agrawal, RM
    Thomas, A
    Brodmerkel, GJ
    GASTROENTEROLOGY, 1998, 114 (04) : A1231 - A1231