Selective Splenic Artery Embolization for the Treatment of Thrombocytopenia and Hypersplenism in Paroxysmal Nocturnal Hemoglobinuria (PNH)

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4408-4408
Author(s):  
Anna Paola Iori ◽  
Karen Brown ◽  
David J. Araten ◽  
Emilia Scalzulli ◽  
Giovanni Fernando Torelli ◽  
...  

Abstract Abstract 4408 PNH is a hemolytic disorder resulting from dysregulation of complement on the rbc, and is associated with immune mediated marrow failure and marked hypercoagulability. Thrombosis can be prevented with anticoagulation or the complement inhibitor eculizumab, and it often involves the hepatic, portal, and splenic veins. This can result in an increase in portal pressure and complications that we term the “Thrombosis-Splenomegaly-Thrombocytopenia” syndrome (TST). TST is frequently associated with abdominal pain, and thrombocytopenia may be simultaneously exacerbated by marrow failure. Particularly, thrombocytopenia can complicate efforts at anticoagulation, leaving the patient exposed to the risk of further events. In some patients thromboses can reversed with tPA, but others will not be candidates because they have presented late after their thromboses. Ablating splenic function would be desirable: however, with a high risk of perioperative thrombosis, surgical splenectomy may be hazardous, particularly in patients who have already had thrombosis, and will confer a risk of sepsis. Here we report on 4 patients with PNH and late-presenting TST. We referred these 4 patients for selective splenic artery embolization (SSAE), which involves cannulating branches of the splenic artery– beyond the hilum– and introducing gelfoam and microcoils. We planned a multi-session stepwise approach: we started with the inferior branches of the splenic artery, to decrease the risk of pleural effusions. We planned to infarct no more than 1/3 of the spleen at any one time and to allow weeks to months for recovery. We routinely administered vaccinations and discontinued anticoagulation temporarily, and patients were given prophylactic antibiotics, fluids, analgesics, and antipyretics, and they were observed in the hospital with a back-up surgical team. Prior to the procedure, the median platelet count was 17 and the median spleen size was 22 cm. Patients 1–4 were treated with 3,2,1, and 3 procedures respectively, which resulted in a significant reduction in spleen volume in all 4 patients. The post procedure platelet counts were respectively 123, 12, 44, and 90, which represented a significant increase for all patients except patient 2, who remained thrombocytopenic; since there was evidence of bone marrow failure, she underwent a successful unrelated SCT. Patients 1,2, and 4 all had had abdominal pain before the procedure, which very significantly improved after recovery from the procedure, which we attribute to decreased venous return from the spleen into the portal circulation. Patients 1–3 were treated before eculizumab was available and patient 1, 3, and 4 are now on it. Patient 4 is a special case in that she had been on eculizumab for several months prior to the SSAE, but had had only a partial reduction in the red cell transfusion requirement; C3d deposition on rbcs was documented by flow cytometry, and it was thought that extravascular hemolysis was limiting her response to eculizumab, as has been described. Of note, her hemoglobin began to rise after the embolization procedure, concurrent with the increase in the platelet count, and a significant further reduction in the red cell transfusion requirement, suggesting that partially reducing splenic function markedly reduced C3-mediated extravascular hemolysis. Of the 9 procedures performed on these 4 patients, only one was complicated by a clinically significant left sided pleural effusion, which was drained by thorascope. All patients are doing well between 3.5 and 11 years after their procedures. We conclude that SSAE, when performed by an experienced interventional radiologist, is relatively safe in patients with PNH and TST, it produces sustained correction of hypersplenism without the risk associated with surgery or the asplenic state, and can be of benefit to patients on eculizumab who have hypersplenism. Pt Age at diagnosis Lowest plt count pre-SSAE Spleen size (cm) pre-SSAE Abdominal pain pre-SSAE Bone marrow Plt count post SSAE Spleen size post SSAE Abdominal pain after recovery from SSAE Complications of SSAE Follow up (yrs) 1 30 42 21 + Hypercellular Megakaryocyte aggregates 123 12 No Abd pain and fever 10 2 18 19 19 + Hypocellular Megas reduced 12 12 No Pain, pleural effusion SCT 3 27 14 36 – Erythroid hyperplasia Megas adequate 44 NA No Abd pain 11 4 26 <10 22 + Normocellular Megas adequate 90 12 No None 3.5 Disclosures: No relevant conflicts of interest to declare.

2018 ◽  
Vol 03 (01) ◽  
pp. 058-061
Author(s):  
Mathew Thomas ◽  
Manish Yadav ◽  
Elsa George

AbstractThe purpose of this pilot study was to assess the effectiveness of partial splenic artery embolization (PSE), in the treatment and management of immune thrombocytopenia (ITP). Six patients with ITP who underwent PSE were followed up. The condition was either refractory to medications like steroids, intravenous immunoglobulin, immunosuppressants, or required very high doses of these drugs which could not be tapered down. Five out of six patients did not have good response even after adding immunosuppresants to steroids. The 6 patients who underwent PSE were followed up to 7 to 18 months with an average duration of 3.5 years. A therapeutic effect was defined as a platelet count of > 10,000/cumm (level below which spontaneous bleeding is likely to occur) at the last follow-up date with marked decrease in drug dosage. A good response was seen in all the patients – their doses of drugs could be reduced considerably and all had platelet count well above 10,000 during the last follow up after PSE. None of the patients who underwent embolization had any serious complications.


2021 ◽  
Vol 2 (2) ◽  
pp. 75-81
Author(s):  
Milica Jeremić ◽  
Danijela Leković ◽  
Dijana Šefer ◽  
Vesna Đorđević ◽  
Andrija Bogdanović

Introduction: Erythrocytosis represents elevated hemoglobin and hematocrit levels above the range of normal values. Primary erythrocytosis - polycythemia vera, is characterized by increased erythrocyte production, due to a disorder at the level of the multipotent stem cell in the bone marrow. On the other hand, secondary erythrocytosis (SE) is the result of bone marrow stimulation by an external factor. Aim: The aim of our study was to determine parameters which are significant in differentiating SE from primary erythrocytosis - polycythemia vera (PV). Materials and methods: This is a retrospective study involving 108 patients with SE and 111 patients with PV, who were diagnosed and treated at the Clinic of Hematology of the Clinical Center of Serbia (CCS), in the period: December 2005 - November 2018.From the patient records, the following data were extracted: demographic characteristics, laboratory parameters, spleen size, total red cell mass, serum erythropoietin (EPO) level, and spontaneous growth of the BFU-E colony. Results: Patients with SE were younger, with a predominance of the male gender and with significantly higher serum EPO values than patients with PV. Patients with PV had significantly higher values of BFU-E, leukocyte and platelet count, spleen size, and LDH level than patients with SE. Total red cell mass analysis did not show a differential diagnostic significance. Conclusion: Findings of normal spleen size, normal leukocyte and platelet count, normal serum LDH level, and elevated EPO, in patients, refer to the diagnosis of secondary erythrocytosis, while the findings of splenomegaly, leukocytosis, thrombocytosis, elevated serum LDH level, decreased EPO, and the presence of spontaneous BFU-E colony speak in favor of the diagnosis of polycythemia vera.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 400-400 ◽  
Author(s):  
Tapani Ruutu ◽  
G. Barosi ◽  
R. J. Benjamin ◽  
R. E. Clark ◽  
J. N. George ◽  
...  

Abstract The pathogenesis of microangiopathy following stem cell transplantation is poorly understood. Endothelial injury is probably an important factor. The spectrum of transplantation-associated microangiopathy (TAM) extends from minor laboratory findings to life-threatening clinical complications. TAM has not been properly defined, a large number of definitions have been used, and the reported incidences and outcomes of this complication have varied greatly. For purposes of clinical study, and to hasten the development of prophylaxis and therapy for this complication, widely accepted and uniform criteria for TAM are essential. An International Working Group was, therefore, formed with the goal of developing a consensus formulation of the criteria necessary for the diagnosis of TAM. Using a mail-only process, 14 experts in microangiopathic disorders and/or transplantation were asked to propose a list of candidate diagnostic criteria, to select those considered necessary, and to rank those considered optional in order to identify a core set of criteria. The three necessary criteria in the core set consisted of: 1)”increased percentage (&gt;4%) of schistocytes in the blood”; 2)”de novo, prolonged, or progressive thrombocytopenia (platelet count ≤50x109/l or a 50% or greater reduction from previous counts)”; and 3)”sudden and persistent increase in LDH”. The four most highly ranked optional criteria were: “decrease in Hb concentration or increased red cell transfusion requirement”, “decrease in serum haptoglobin”, “sudden and persistent increase in BUN or creatinine”, and “neurological symptoms”. In an appropriateness panel process, the experts were then asked to score the diagnosis of 16 patient profiles as appropriate or not appropriate for TAM. Using the experts’ consensus, the performance (sensitivity and specificity) of 24 possible definitions of the disorder obtained from the core set of criteria was evaluated. The definition of TAM with the highest final score was: the three necessary criteria, plus decrease in Hb concentration or increased red cell transfusion requirement plus decrease in serum haptoglobin. The Working Group, consequently, proposes that the diagnosis of TAM requires fulfilment of all of the following criteria: 1) increased percentage (&gt;4%) of schistocytes in the blood; 2) de novo, prolonged, or progressive thrombocytopenia (platelet count ≤50x109/l or 50% or greater reduction from previous counts); 3) sudden and persistent increase in LDH; 4) decrease in Hb concentration or increased red blood cell transfusion requirement; and 5) decrease in serum haptoglobin. This definition has &gt;80% sensitivity and specificity.


2019 ◽  
Author(s):  
Amit Kumar Verma ◽  
Stephen Edward Ryan ◽  
Ashish Gupta ◽  
Adnan Hadziomerovic ◽  
Karl Smyth ◽  
...  

2021 ◽  
Vol 38 (01) ◽  
pp. 105-112
Author(s):  
Majd Habash ◽  
Darrel Ceballos ◽  
Andrew J. Gunn

AbstractThe spleen is the most commonly injured organ in blunt abdominal trauma. Patients who are hemodynamically unstable due to splenic trauma undergo definitive operative management. Interventional radiology plays an important role in the multidisciplinary management of the hemodynamically stable trauma patient with splenic injury. Hemodynamically stable patients selected for nonoperative management have improved clinical outcomes when splenic artery embolization is utilized. The purpose of this article is to review the indications, technical aspects, and clinical outcomes of splenic artery embolization for patients with high-grade splenic injuries.


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