scholarly journals Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Behyar Zoghi ◽  
Paul Shaughnessy ◽  
Roger M. Lyons ◽  
Richard Helmer ◽  
Carlos Bachier ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Stem Cell ◽  
Stem Cell Transplant ◽  
Significant Risk ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Cell Transplant ◽  
Hematopoietic Stem ◽  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand

The use of high dose chemotherapy followed by autologous hematopoietic stem cell transplantation for remission consolidation after initial induction represents standard of care for patients with multiple myeloma. Patients with myeloma and Acquired von Willebrand Syndrome (AVWS) undergoing autologous stem cell transplant (ASCT) are at significant risk of bleeding due to the profound thrombocytopenia, low Factor VIII levels, fever, and toxicities associated with the preparative regimen. We report a patient with AVWS associated with multiple myeloma who underwent autologous stem cell transplants as consolidation after initial induction and again at relapse. He was successfully treated with high dose intravenous immunoglobulin (IVIG) prior to each transplant with rapid resolution of AVWS.

Acquired von Willebrand syndrome in association with multiple myeloma: remission after stem cell transplant

2021 ◽  
Vol 14 (1) ◽  
pp. e239053
Author(s):  
Chintan P Shah ◽  
Jess Delaune ◽  
Molly W Mandernach
Keyword(s):  
Multiple Myeloma ◽  
Stem Cell ◽  
Myeloproliferative Neoplasm ◽  
Significant Risk ◽  
Coagulation Factor ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Cell Transplant ◽  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand

Acquired von Willebrand syndrome is a rare bleeding disorder characterised by a later age of onset without a personal or family history of bleeding diathesis. It is vital to discern acquired von Willebrand syndrome from inherited von Willebrand disease and other acquired bleeding disorders as management differs significantly. Acquired von Willebrand syndrome is usually secondary to an underlying disorder such as lymphoproliferative disorder, myeloproliferative neoplasm, solid tumour, cardiovascular disorder, autoimmune disorders or hypothyroidism. Diagnosis is often delayed with a significant risk of morbidity and even mortality. Here we present a case of a 74-year-old man with an acquired bleeding disorder and work up suggestive of acquired von Willebrand syndrome secondary to immunoglobulin G kappa multiple myeloma. He was treated successfully with intravenous immunoglobulin, von Willebrand Factor/Coagulation Factor VIII Complex (human), myeloma directed chemotherapy and autologous stem cell transplantation. We also discuss the management strategies that are largely based on retrospective studies and case reports.

Incidence of Adrenal Insufficiency in Patients with Multiple Myeloma during High Dose Chemotherapy and Autologous Stem Cell Transplant

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5862-5862 ◽  
Author(s):  
Ahmad Hatem Mattour ◽  
Shatha Farhan ◽  
Nalini Janakiraman ◽  
Edward Peres
Keyword(s):  
Multiple Myeloma ◽  
Stem Cell ◽  
Adrenal Insufficiency ◽  
Stem Cell Transplant ◽  
High Dose Chemotherapy ◽  
Autologous Stem Cell Transplant ◽  
Adrenal Function ◽  
High Dose ◽  
Cell Transplant ◽  
Cortisol Levels

Abstract Background High dose chemotherapy (HDC) followed by autologous stem cell transplant (ASCT) is considered the standard of care for patients with multiple myeloma (MM). With most patients receiving induction therapy that includes corticosteroids. The combined effect of prior therapy for myeloma and ASCT related complications may result in hypotension and require intensive medical treatment. To date the incidence of adrenal insufficiency in the setting of ASCT is unknown. The effects of this underlying disorder in regards to post transplant outcome remain unknown as well. We set out to compare the outcomes of patients with multiple myeloma who underwent ASCT with adrenal insufficiency compared to those with sufficient adrenal function. Methods We undertook a prospective study in 13 consecutive patients with Multiple Myeloma admitted for high dose chemotherapy and autologous stem cell transplant at Henry Ford Hospital between February 2014 through June 2014, with the first patient sample being obtained in on 2/14/2014 and last sample recorded in 6/23/2014. Random cortisol levels were obtained on the day of admission or day -2 (figure 1), prior to the start of high dose chemotherapy. All prior therapies included corticosteroids and consisted of the following RVD, DCEP, RD, VD, DT-PACE. Patients were classified into two groups those with cortisol levels > 5 and those patients who had cortisol levels <5. Endpoints analyzed included hypotension, septic shock, and duration of antibiotic therapy. Results Of the 13 patients analyzed the median age was 60 years old (range 53-78), gender 8 male patients 5 female. All patients underwent high dose chemotherapy with Melphalan and Autologous stem cell transplant. Of the 13 patients in which data was obtained 3/13 23% had adrenal insufficiency prior to high dose Melphalan and ASCT. With a median cortisol level of 3.2 in the patient cohort who were found to be adrenal insufficient. The incidence of hypotension was 2/3 66% in the adrenal insufficient patients compared 1/10 10% in the cohort with sufficient adrenal function. Septic shock occurred in 2/3 66% of the adrenal insufficient compared to 0/10 in the adrenal sufficient group. The median duration of antibiotic therapy was 5 days in the adrenal insufficient cohort compared to 2 days in the patients with adequate adrenal function. Conclusion In this small cohort of consecutive patients from a single center, we found that there was a high incidence of adrenal insufficiency 23% in patients undergoing ASCT. The treatment regimens varied in the study group with all patients receiving corticosteroid therapy in there induction regimen. Given the unexpectedly high incidence of adrenal insufficiency a consideration should be given to check a random cortisol level prior to the initiation of HDC. Supplemental therapy or treatment should be considered in this high risk group to avoid unnecessary complications and prolonged antibiotic use and supportive care in this patient cohort. Figure 1. Random Cortisol levels obtained prior to Autologous stem cell transplant Figure 1. Random Cortisol levels obtained prior to Autologous stem cell transplant Disclosures No relevant conflicts of interest to declare.

Response Before Autologous Hematopoietic Stem Cell Transplantation Is An Important Predictor of Outcome in Multiple Myeloma,

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4119-4119
Author(s):  
Shatha Farhan ◽  
Heather Lin ◽  
Veerabhadran Baladandayuthapani ◽  
Nina Shah ◽  
Qaiser Bashir ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Stem Cell ◽  
Partial Response ◽  
Hematopoietic Stem Cell ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Cell Transplant ◽  
Minor Response ◽  
Hematopoietic Stem ◽  
The Impact

Abstract Abstract 4119 Background: The outcome of multiple myeloma (MM) has improved significantly over the last 2 decades due to the availability of several novel agents and an increasing use of high-dose chemotherapy and autologous hematopoietic stem cell transplant (auto-HCT). There is considerable evidence that the response to induction therapy predicts a favorable outcome after high-dose chemotherapy and auto-HCT. In this report, we evaluated the impact of pre-transplant response to conventional agents on post auto-HCT outcomes including time to progression (TTP), progression free survival (PFS) and overall survival (OS). Methods: 1567 patients received auto-HCT at UT-MD Anderson Cancer Center between 1988 and December 2010. Pre auto-HCT disease status was divided into the following prior auto-HCT response groups: complete response (CR), very good partial response (VGPR), partial response (PR), minor response (MR), stable disease (SD) and progressive Disease (PD). The primary objective was to study the impact of prior response on the outcome. Demographics, disease-related and transplant-related variables were collected. The distributions of TTP, PFS and OS were estimated by the Kaplan-Meier method. Log-rank test was performed to test the difference in survival between groups. Regression analyses of survival data was based on the univariate and multivariate Cox proportional hazards model. Results: Median age at auto-HCT was 56.8 years. Median time from diagnosis to auto-HCT was 7.9 months. Of the 1567 patients, 72 (4.6%) achieved CR before auto-HCT, 255 (16%) had VGPR, 848 (54%) had PR, 78 (5%) had MR, 189 (12%) had SD, 88 (5.6%) had PD, while the response was unknown in 37 (2%) patients. Almost 17% patients were 65 years or older at auto-HCT and approximately 7% had high-risk cytogenetic abnormalities. On univariate analysis, the response prior to auto-HCT had significant impact on TTP (p=<0.0001), PFS (p=<0.0001), and OS (p=<0.0001), In multivariate models, the response prior to auto-HCT still emerged as a significant predictor of TTP, PFS and OS (p =0.0055, p=0.0004 and p<0.0001, respectively) after adjusting for percent of plasma cells, time interval from diagnosis to transplantation and/ or creatinine level. In particular, patients who achieved CR prior to auto-HCT had significantly longer TTP, PFS and OS when compared to patients who had PR, MR, SD or PD (Figures 1–Fig 3). In contrast, TTP, PFS and OS were not significantly different between patients who achieved a CR or VGPR prior to auto-HCT. Conclusion: Patient who achieved CR or VGPR before undergoing an auto-HCT had significantly longer TTP, PFS and OS after the transplant. These results highlight the importance of effective anti-myeloma therapy to maximize the response before proceeding to auto-HCT. Disclosures: No relevant conflicts of interest to declare.

scholarly journals EXPRESS: Pulmonary Arterial Hypertension in Pediatric Patients Undergoing High Dose Carboplatin/Thiotepa and Stem Cell Transplant

2021 ◽  
pp. 204589402110504
Author(s):  
Gretchen Louise Hackett ◽  
Farrah Munir ◽  
Teresa Shapiro ◽  
Robert Greiner ◽  
Daniel J. McKeone ◽  
...  
Keyword(s):  
Stem Cell ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Stem Cell Transplant ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Cell Transplant ◽  
Hematopoietic Stem ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) in pediatric patients is associated with significant morbidity and mortality. Few studies exist to evaluate the incidence of PAH in pediatric oncology patients treated with carboplatin and thiotepa followed by hematopoietic stem cell transplant (HSCT). We describe two pediatric patients who developed PAH following high dose chemotherapy with carboplatin and thiotepa followed by autologous HSCT. These cases highlight the need for a surveillance protocol and reinforce the need for cardiologist-oncologists or close collaboration between providers in these sub-specialties who provide care to patients undergoing this type of chemotherapy regimen.

A Comparative Study Of The Outcome Of Isolated Chromosome 13q and Clonal Progression Detected By I-FISH Do Additional Cytogenetic Abnormalities Impact Survival In Multiple Myeloma

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2160-2160
Author(s):  
Edward Peres ◽  
Shatha Farhan ◽  
Philip Kuriakose ◽  
Susan Michalowski ◽  
Alexandra Sitarik ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Overall Survival ◽  
Stem Cell ◽  
Stem Cell Transplant ◽  
High Dose Chemotherapy ◽  
Autologous Stem Cell Transplant ◽  
High Dose ◽  
Cell Transplant ◽  
Cytogenetic Abnormalities ◽  
Chromosome 13Q

Abstract Background Chromosomal abnormalities detected by interphase fluorescence in situ hybridization (I-FISH) are an important prognostic marker in patients with multiple myeloma (MM). Isolated chromosome 13q has been considered standard risk when identified by I-FISH and high risk by conventional cytogenetics. The impact of additional cytogenetic abnormalities with chromosome 13q identified by I-FISH in regards to prognosis has not been fully defined. In this report, we describe the outcome of patient’s with multiple myeloma with isolated chromosome 13q and 13q+ (additional cytogenetic abnormalities) identified by I-FISH at our institution between January 2003 and January 2013 and had I-FISH analysis prior to treatment. Methods The primary objective was to compare patient’s outcomes in regards to response, time to progression, and overall survival between patients who had an isolated 13q and 13q+ identified by I-FISH in the bone marrow plasma cells. Kaplan & Meier curves were generated to calculate overall survival (OS) between the two groups. Results Between January 2003 and January 2013, we identified 76 patients by I-FISH who had either an isolated 13q or 13q+ in patients with multiple myeloma (Patient characteristics Table 1). Of the patients with an isolated 13q abnormality 33% received a bortezomib-based regimen and 38% in the 13q+ group. Of the patient’s with a isolated 13q 38% went onto receive high dose chemotherapy followed by autologous stem cell transplant (ASCT) while 20% with a 13q+ received ASCT. African American patients with 13q consisted of 65% and 60% with 13q+ in our patient population. For the 13q or 13q+ who underwent high dose chemotherapy followed by autologous stem cell transplant OS was 85% compared to the non-transplant group 45% (p=0.01) (Figure 2). On follow up at a median of 2.5 years mortality occurred in 31% of the 13q patients compared to 62% in the 13q+ group. The overall survival at 5 years was 25% in the 13q+ group compared to 65% in the patient’s with an isolated 13q, With the 13q+ group having an overall poor OS (p=0.03) Conclusion Patients who harbor the 13q and additional cytogenetic abnormalities identified by I-FISH have a significant worse outcome compared to patients with an isolated 13q. These patients should be considered high risk and consideration for treatment with novel agents and autologous stem cell transplant followed by post-transplant maintenance therapy should be considered. Disclosures: No relevant conflicts of interest to declare.

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