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
  • [31] Efficacy of Partial Splenic Embolization in the Management of Refractory Immune Thrombocytopenia
    Singh, Avani M.
    Ionescu, Filip
    Jaglal, Michael
    Kis, Bela
    Eatrides, Jennifer
    BLOOD, 2022, 140 : 11255 - 11256
  • [32] Percutaneous Partial Splenic Embolization for Thrombocytopenia Following Liver Transplantation
    Amesur, N. B.
    Zajko, A. B.
    Fontes, P.
    Sharma, V
    AMERICAN JOURNAL OF ROENTGENOLOGY, 2007, 188 (05)
  • [33] Partial splenic embolization in the treatment of thrombocytopenia after liver transplantation
    Herrero, JI
    Sangro, B
    Quiroga, J
    Bilbao, JI
    Yuste, JR
    Longo, J
    Pardo, F
    Hernandez, JL
    Cienfuegos, JA
    Prieto, J
    TRANSPLANTATION, 1997, 63 (03) : 482 - 484
  • [34] Partial splenic embolization in myelodysplastic syndrome associated with immune thrombocytopenia
    Ozdogu, H
    Boga, C
    Oguzkurt, L
    Kizilkilic, E
    JOURNAL OF THROMBOSIS AND THROMBOLYSIS, 2005, 18 (03) : 213 - 216
  • [35] THE EFFECT OF PARTIAL SPLENIC EMBOLIZATION FOR THROMBOCYTOPENIA IN LIVER CIRRHOSIS PATIENTS
    Kim, B. I.
    Cho, Y. K.
    Kim, H. J.
    Park, J. H.
    Park, D. I.
    Sohn, C. I.
    Jeon, W. K.
    Hong, H. P.
    JOURNAL OF HEPATOLOGY, 2012, 56 : S250 - S250
  • [36] Partial Splenic Embolization in Myelodysplastic Syndrome Associated with Immune Thrombocytopenia
    Hakan Ozdogu
    Can Boga
    Levent Oğuzkurt
    Ebru Kı zı lkı lı c
    Journal of Thrombosis and Thrombolysis, 2004, 18 : 213 - 216
  • [37] EFFICACY OF ENZYME REPLACEMENT THERAPY WITH VELAGLUCERASE ALFA IN PATIENTS WITH TYPE 1 GAUCHER DISEASE AND THROMBOCYTOPENIA OR SEVERE SPLENOMEGALY
    Giraldo, P.
    Gonzalez, D.
    Kabra, M.
    Lukina, E.
    Kisinovsky, I.
    Elstein, D.
    Zahrieh, D.
    Crombez, E.
    Zimran, A.
    HAEMATOLOGICA, 2013, 98 : 196 - 197
  • [38] Partial Splenic Embolization for the Management of Severe Refractory Thrombocytopenia in Antiphospholipid Syndrome: A Case Report and Literature Review
    Jalili, Javad
    Pourghorban, Ramin
    Mahmoudpour, Masoud
    Akhavi Milani, Ali
    VASCULAR AND ENDOVASCULAR SURGERY, 2022, 56 (03) : 316 - 320
  • [39] Gaucher's Disease, an Unusual Cause of Massive Splenomegaly, a Case Report
    Binesh, F.
    Yousefi, A.
    Ordooei, M.
    Bagherinasab, M. A.
    IRANIAN JOURNAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY, 2013, 3 (04) : 173 - 175
  • [40] Case Report: Splenic Artery Aneurysm and Splenomegaly in Gaucher Disease during Pregnancy
    Radakovic, Trsnjak A.
    Schulte, C.
    Klein, I
    Peter, D.
    Lichthardt, S.
    Reibetanz, J.
    Nothhaft, M.
    Woeckel, A.
    Lund, H.
    Rehn, M.
    GEBURTSHILFE UND FRAUENHEILKUNDE, 2022, 82 (06) : 635 - 635