Successful use of anti-CD20 (rituximab) in severe, life-threatening childhood immune thrombocytopenic purpura

2003 ◽  
Vol 143 (5) ◽  
pp. 670-673 ◽  
Author(s):  
Kristi L Bengtson ◽  
Michael A Skinner ◽  
Russell E Ware
CHEST Journal ◽  
2016 ◽  
Vol 150 (4) ◽  
pp. 392A ◽  
Author(s):  
Gautam Sikka ◽  
Aditya Gupta ◽  
Moses Bachan ◽  
Zinobia Khan

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4996-4996 ◽  
Author(s):  
Elizabeth A. Guancial ◽  
Vinay S. Mahajan ◽  
Ronald P. McCaffrey ◽  
Neal Lindeman

Abstract Abstract 4996 The application of monoclonal antibodies represents an increasingly powerful weapon in the arsenal against hematologic diseases. With the advent of new therapies come previously unrecognized laboratory interference that if not properly identified could have clinical repercussions. We encountered two patients, neither of whom had a history of plasma cell dyscrasia, who underwent treatment with rituximab for immune thrombocytopenic purpura (ITP) and were discovered to have a monoclonal IgG kappa band on serum protein electrophoresis (SPEP) followed by immunofixation (IFX). Both patients had severe, steroid-resistant ITP and received rituximab on an empiric basis, at a weekly dose of 375 mg/m2 IV. In Patient #1, an evaluation for secondary causes of thrombocytopenia included SPEP and IFX. The IFX yielded a faint, atypical IgG kappa band that migrated to the far cathodal zone of the gel. No band was seen on SPEP alone. Since the patient was treated with rituximab the day before these studies were performed, we considered the possibility that the IgG kappa band was due to circulating rituximab. Rituximab is a chimeric antibody composed of human IgG1 kappa chain constant regions and heavy- and light-chain variable regions from a murine anti-CD20 antibody. The elimination half-life of this therapeutic antibody increases with subsequent doses and is approximately 60 hours for the first dose and 174 hours for the fourth dose (range 26 to 442 hours). An aliquot of rituximab was obtained from pharmacy and analyzed by SPEP/IFX, showing an IgG kappa monoclonal protein band identical to the M-protein in the patient's serum. Repeat assessment of serum from this patient obtained immediately prior to her fourth treatment with rituximab demonstrated that the IgG kappa paraprotein was less intense on IFX compared to the first IFX. Several hours after the fourth rituximab infusion was completed, a more intense band corresponding to the IgG kappa paraprotein was detected again on IFX. In Patient #2, who also received rituximab for steroid-resistant ITP, a similar circulating rituximab-related protein was detected on IFX performed one hour after his fourth rituximab infusion. IFX performed on a sample obtained immediately before treatment did not demonstrate a paraprotein, and testing one week after treatment showed that the band had resolved. We believe that awareness of the presence of a faint monoclonal protein by SPEP/IFX following recent administration of rituximab is important to avoid unnecessary further evaluation for a pathologic monoclonal gammopathy. Alternatively, if suspicion exists, SPEP/IFX should be conducted prior to the initiation of treatment with rituximab or after the treatment course is completed in order to avoid uncertainty. Furthermore, it is important to be cognizant of this finding in patients on maintenance rituximab for an indolent lymphoma and an associated paraprotein who may undergo interval monitoring of the monoclonal gammopathy. It is possible that a similar phenomenon may be seen with other therapeutic monoclonal antibodies with a dosing regimen and half-life that is similar to rituximab, such as cetuximab, a monoclonal antibody directed against the epidermal growth factor receptor. Finally, the presence of a characteristic band on immunofixation may allow for the qualitative assessment of the presence of a therapeutic monoclonal antibody in the serum. Further studies are necessary to determine the sensitivity and specificity of such a laboratory test. Disclosures: Off Label Use: Rituximab is an anti-CD20 chimeric antibody and is used for the treatment of immune thrombocytopenic purpura.


2020 ◽  
Vol 4 (3) ◽  
pp. 421-423
Author(s):  
Melanie Randall ◽  
Jason Nurse ◽  
Karan Singh

Introduction: Immune thrombocytopenic purpura (ITP) is an autoimmune-mediated disorder in which the body produces antibodies that destroy platelets, causing an increased risk of bleeding and bruising. Tranexamic acid (TXA) is a medication that prevents clot breakdown and is used to treat uncontrolled bleeding. Case Report: We present the case of an 11-year-old female with significant epistaxis and hypotension in the emergency department. Traditional therapies were initiated; however, the patient continued to have bleeding and remained hypotensive, so intravenous TXA was given. The patient’s bleeding then resolved. Conclusion: TXA may be a safe and effective adjunct to traditional therapies for the treatment of life-threatening hemorrhage in ITP patients.


2020 ◽  
Author(s):  
Valérie Lévesque ◽  
Émilie Milaire ◽  
Daniel Corsilli ◽  
Benjamin Rioux-Massé ◽  
François Martin Carrier

Abstract Purpose: COVID-19 is a new disease with many undescribed clinical manifestations. Material and methods: We report herein a case of severe immune thrombocytopenic purpura (ITP) in a critical COVID-19 patient.Results: A patient presented a severe episode of immune thrombocytopenia (< 10 x 109/L) 20 days after admission for a critical COVID-19. This thrombocytopenia was associated with a life-threatening bleeding. Response to first-line therapies was delayed as it took up to 13 days after initiation of intravenous immunoglobulin and high dose dexamethasone to observe an increase in platelet count. Conclusion: COVID-19 may be associated with late presenting severe ITP. Such ITP may also be relatively resistant to first-line agents. Hematological manifestations of COVID-19, such as the ones associated with life-threatening bleeding, must be recognized.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 2074-2074
Author(s):  
JRafael Cabrera ◽  
F. Javier Penalver ◽  
Isabel Millan ◽  
Victor Jimenez-Yuste ◽  
Manuel Almagro ◽  
...  

Abstract Objectives: Rituximab (RB) is an anti-CD20 murine/human chimeric antibody that has been used in the treatment of auto-immune diseases, including immune thrombocytopenic purpura (ITP). The effectiveness of RB in the treatment of patients with ITP has been evaluated. Patients and methods: A multicentric retrospective study of the patients with ITP treated with RB has been carried out. A questionnaire about clinical characteristics and response to RB was sent to the participating centers. These questionnaires were filled out by the physicians in charge of each patient. We received questionnaires from 43 centers The response criteria were those published by Stasi R et al (Blood2001; 98:952): Complete Response (CR): platelets >100 x 109/l; Partial Response (PR): 50–100 x 109/l; Minimum Response (MR): 30–50 x 109/l. Data from 99 patients were analyzed. Ninety two out of 99 patients were evaluated. The median age at diagnosis was 54 years (4–98), 56% were women. 55.4% of the cases were idiopathic ITP, 14.1% were associated with B-CLL, 8.7% with auto-immune diseases and 3.3% with lymphoma. Before RB they have received several treatment schemes including steroids (98%), IVIG (88%) and cyclophosphamide (26%). 52.2% were splenectomized. Median of platelets before treatment with RB was 8 x 109/l (1–30), 66.3% had less than 10 x 109/l. 87% of the patients were treated with 4 doses of RB (1–6), while 34 received it in association with other treatments, mainly steroids. Results: 42 patients obtained CR (46.2%) and 8 patients obtained PR (8.8%), a total of 50 global responses (55%). The majority of the responses took place in the first week (26% out of the total, 40% of the responders). The maximum response was also soon achieved, median of 5 weeks, with platelets of 141 x 109/l (10–651). There were no differences in the response between splenectomized or not splenectomized patients. 29 patients maintained the CR (69% of responders), with a monitoring median of 9 months (2–42). The toxicity of the treatment was minimum: 2 fever episodes with the infusion and 2 skin eruptions. Conclusions: This is the widest series of patients with ITP treated with RB. These results suggest that RB can be considered as a rescue treatment for patients with refractory ITP to steroids and/or splenectomy, with an excellent tolerance. Further monitoring is needed in order to establish the real role of RB as a rescue treatment for refractory ITP.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3977-3977 ◽  
Author(s):  
Delvyn C. Case ◽  
Jacquelyn A. Hedlund ◽  
Kurt S. Ebrahim ◽  
Marjorie A. Boyd

Abstract ITP in adults is an autoimmune disease characterized by antibody-mediated thrombocytopenia. A significant number of patients relapse after initial therapy with prednisone and splenectomy when necessary, and require other therapy for continuing thrombocytopenia. Rituximab, an anti-CD20 chimeric monoclonal antibody has been utilized in ITP refractory to other modes of treatment (Feurestein, M., et al. The use of Rituximab in 28 patients with immune thrombocytopenic purpura (ITP). Proc. Am. Soc. Clin. Onc.22:187, 2003). We report on the long-term responses possible in ITP treated with Rituximab. Twenty-two patients with ITP refractory to initial conventional therapies with platelet counts &lt;30,000/ul were treated with Rituximab 375 mg/m2 IV weekly x4. Responses were characterized as partial response (PR; platelets &gt;50,000/ul but &lt;150,000/ul) and complete response (platelets &gt;150,000/ul). Patient characteristics included 5 males and 17 females with ages 24–83 years (median 58). Twenty had undergone splenectomy. Thirteen patients (59%) responded to Rituximab. Six responses were PR with durations lasting 2, 2, 3, 3, 4, and 6 months. Continuing Rituximab monthly after initial therapy of 4 weeks did not produce improved platelet counts in patients who failed Rituximab or who achieved PR. Seven responses were CR with durations of 12, 20+, 25+, 29+, 38+, 40+, and 48+ months. The one patient who relapsed at 12 months was retreated with Rituximab but did not respond. There was no correlation between response and age, sex, or duration of ITP. Neither of the patients who declined prior splenectomy responded. There were no serious complications of Rituximab infusions. Twenty-five percent of patients had mild first infusion reactions. Rituximab can produce long-term CR in 27% of patients with ITP refractory to initial therapy.


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