scholarly journals Treatment Reality of Patients with Immune Thrombocytopenia (ITP) in Routine Care

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5009-5009
Author(s):  
Rudolf Weide ◽  
Stefan Feiten ◽  
Vera Friesenhahn ◽  
Jochen Heymanns ◽  
Kristina Kleboth ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Treatment Guidelines ◽  
Conflicts Of Interest ◽  
Treatment Options ◽  
Immunosuppressive Agents ◽  
Routine Care ◽  
Treatment Modalities ◽  
Bleeding Complications ◽  
Line Therapy

Abstract Introduction: New treatment options like thrombopoietin receptor agonists (TRAs) and rituximab have been introduced into the clinic which have found their way into national and international treatment guidelines. The aim of this study was to answer the following questions concerning diagnosis and treatment of patients with ITP in routine care: How are patients diagnosed and treated?Which sequences of therapy are applied?How many patients die due to bleeding complications? Methods: All patients with ITP diagnosed between 06/1995-06/2014 in a community-based oncology group practice in Germany were analyzed retrospectively. Results: 402 patients with a median age of 55 (7-90) were evaluated. 57% were female and 43% male. 357 (89%) were classified as primary ITP and 311 (77%) as having chronic ITP. In 234 patients (58%) a bone marrow biopsy was part of the diagnostic work up. Only 191 patients (48%) needed therapy. First line therapy (n=191) were steroids in 81%, intravenous immunoglobulins (ivIgG) in 12% and ivIgG plus steroids in 6%. Second line therapy (n=102) were ivIgG in 49%, steroids in 23%, ivIgG plus steroids in 17%, other immunosuppressive agents in 11% and splenectomy in 5%. Third line therapy (n=63) was splenectomy in 22%, other immunosuppressive agents in 27%, steroids in 19%, ivIgG in 16%, 11% combination therapy, rituximab in 10% and TRAs in 5%. Fourth line therapy (n=38) consisted of steroids in 26%, splenectomy in 26%, immunosuppressive agents in 34%, ivIgG in 13%, rituximab in 11% and TRAs in 3%. Patients received a median of 2 lines (1-10) of therapy. Treatment modalities most frequently used were steroids in 93%, immunoglobulins in 56%, splenectomy in 21% and other immunosuppressive agents in 21% of patients. Rituximab and TRAs were used in 10% and 5% only. 75% of patients received a durable remission (complete or partial) after their last therapy. 10% showed no response, in 15% remission couldn't be evaluated due to external treatments. 146 patients (76%) are off treatment. 1 patient (0.2%) died due to bleeding complications. Conclusions: Bone marrow biopsy is used as a diagnostic procedure in 58% of patients. Treatment modalities most frequently used are steroids, immunoglobulins, splenectomy and other immunosuppressive agents. Rituximab and TRAs are used infrequently. A high percentage of ITP-patients achieve durable remissions and ITP-related mortality is low. Disclosures No relevant conflicts of interest to declare.

Successful Treatment of Acquired Amegakaryocytic Thrombocytopenia with Rituximab: A Case Report.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4223-4223
Author(s):  
Laura R. Goldberg ◽  
Lewis Glasser ◽  
Tony Wu ◽  
Ikue Shimizu ◽  
Peter J. Quesenberry ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Case Report ◽  
Bone Marrow Biopsy ◽  
Conflicts Of Interest ◽  
Immunosuppressive Agents ◽  
Peripheral Blood Smear ◽  
Severe Thrombocytopenia ◽  
Literature Report ◽  
Platelet Counts ◽  
Acquired Amegakaryocytic Thrombocytopenia

Abstract Abstract 4223 Acquired amegakaryocytic thrombocytopenia is a rare disorder in which there is a marked decrease in bone marrow megakaryocytes leading to severe thrombocytopenia with preserved hematopoeisis in the remaining lineages. The clinical course is variable and no standardized treatment exists. Multiple cases in the literature report treatment using immunosuppressive agents including cyclosporine and antithymocyte globulin. In this case report, we describe the first successful use of rituximab in a patient with amegakaryocytic thrombocytopenia in the absence of any underlying autoimmune disorders. Our patient, an 86- year-old woman, presented with epistaxis, ecchymoses, and blood-tinged sputum. She had thrombocytopenia (platelets 6 × 109/L, MPV 8.3) with a normal hemoglobin and white blood cell count. Peripheral blood smear showed no obvious cause for thrombocytopenia. Bone marrow biopsy revealed focal lymphocytic infiltrates and severe megakaryocytic hypoplasia with nearly absent megakaryocytes. Cytogenetic and molecular analyses were normal, revealing no evidence of a clonal myelo- or lymphoproliferative disorder. The diagnosis of amegakaryocytic thrombocytopenia was made. She was started on prednisone 50mg per day. There was no significant improvement in her platelet count and she was then given IVIG without response. Repeat bone marrow biopsy showed persistent severe bone marrow hypoplasia with near absence of megakaryocytes. After 6 weeks of platelet transfusion dependence and steroids, she was tapered off her steroids and was given rituximab 375mg/m2 weekly for 4 doses. Three weeks after starting rituximab, her platelet counts began to recover and by day 37, her platelet counts normalized and remained within normal limits 12 months after treatment. This case demonstrates the possible utility of rituximab in treating patients with isolated acquired amegakaryocytic thrombocytopenia. Disclosures: No relevant conflicts of interest to declare.

scholarly journals The Platelet Millionaire: A Rare Cause of Thrombocytosis in an Adolescent

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5391-5391
Author(s):  
Ritika Walia ◽  
Theresa Sepulveda ◽  
Sharon Wretzel ◽  
Philip H Brandt
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Primary Care Physicians ◽  
Conflicts Of Interest ◽  
Abdominal Ultrasound ◽  
Cell Transplant ◽  
Peripheral Smear ◽  
Hematopoietic Stem ◽  
Marrow Fibrosis

Objectives: Primary myelofibrosis is rare in pediatrics, often manifesting as persistent idiopathic thrombocytosis.Transitions from pediatric to adult medical care can be complicated by workup requiring invasive procedures. J.M., an 18-year-old healthy male, presented for excessive gingival bleeding after wisdom tooth extraction. Workup revealed persistent thrombocytosis to 1,165K, prompting a referral to hematology-oncology. A peripheral smear was notable for many platelets but normal RBC morphology. He had splenomegaly on abdominal ultrasound and a decreased von-Willebrand's activity to antigen ratio, suggesting acquired vWD. A bone marrow biopsy was advised; however, J.M. became lost to follow up for over 9 months owing to self-reported anxiety about the procedure. He remained asymptomatic in this interim until he re-presented to clinic for easy bruising, with no other evidence of bleeding at the time. The biopsy was pursued, revealing hypercellular marrow for age with left shifted granulocytic and erythroid maturation, abnormal megakaryocytes, and 3% blasts. This was consistent with primary early myelofibrosis (PMF), positive for MF-1, CALR, and TP53 mutations and negative for JAK2 and BCR-ABL. He was transitioned to adult hematology, maintained on baby aspirin, and referred for potential allogeneic hematopoietic stem cell transplant (HSCT). PMF is characterized by marrow fibrosis due to secretion of fibroblast growth factor by clonally proliferative megakaryocytes. It is a disease of adulthood, with 67 years being the median age at diagnosis. Only 100 cases have been reported in children, most of which are secondary to AML, ALL or other malignancies.1 Most patients present with complications of extramedullary hematopoiesis or bleeding.2 Diagnosis is suggested by a leukoerythroblastic picture on peripheral smear and confirmed with a bone marrow biopsy "dry tap" revealing marrow fibrosis.3 Prognosis in pediatric PMF is difficult to predict but outcomes tend to be worse;4 TP53 mutation is rare and based on limited adult studies may portend a poorer prognosis.5 Our young patient with this rare mutation was therefore referred for HSCT evaluation. Further complicating this case was J.M.'s anxiety, which delayed definitive diagnosis by biopsy. He only agreed to it when, at the med-peds clinic, the concept of local pain management was discussed. Anticipation of upcoming procedures by primary care physicians and close follow-up is especially important for patients transitioning from pediatric to adult providers. Disclosures No relevant conflicts of interest to declare.

The Certainty of a Post-Bone Marrow Diagnosis: A Study of the Yield of Bone Marrow Biopsies in a Community Hospital Setting

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 34-35
Author(s):  
Manasi M. Godbole ◽  
Peter A. Kouides
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Fever Of Unknown Origin ◽  
Conflicts Of Interest ◽  
Diagnostic Yield ◽  
Hospital Setting ◽  
Unknown Origin ◽  
Nutritional Deficiencies ◽  
Bone Marrow Biopsies

Introduction: Most studies on the diagnostic yield of bone marrow biopsy including the one by Hot et al. have focused on the yield of bone marrow biopsies in diagnosing the source of fever of unknown origin. However, there have not been any studies performed to our knowledge looking at overall practice patterns and yield of bone marrow biopsies for diagnoses other than fever of unknown origin. We aim to determine the most common indications for performing bone marrow biopsies in a community-based teaching hospital as well as the yield of the biopsies in patients with specified and unspecified pre-test indications to estimate the rate of uncertain post-test diagnoses. Methods: We performed a retrospective data collection study at Rochester General Hospital, NY. A comprehensive search was conducted in our electronic medical data to identify all patients who underwent bone marrow biopsies over a 5 year period from January 2011 - December 2016 for indications other than fever of unknown origin. Patient data including demographics, pre-bone marrow biopsy diagnosis and post-bone marrow diagnosis was obtained. All patients above the age of 18 who underwent bone marrow biopsy for indications other than fever of unknown origin or follow up treatment of a hematological malignancy were included. Results: A total of 223 biopsies were performed. The median age was 59 years (age range- 23-95). One hundred and sixteen patients were male and 107 were female. The most common indications for performing bone marrow biopsy were evaluation of the following possible conditions: multiple myeloma (n=54), myelodysplastic syndrome [MDS] (n=47), lymphoma (n=28) and leukemia (n=18) as well as non-specific indications such as pancytopenia (n=40), anemia (n=22) and thrombocytopenia (n=11). The proportion of cases confirmed by bone marrow biopsy was 45/54 (83%) with the pre-marrow diagnosis of multiple myeloma, 34/47 cases (72%) with the pre-marrow diagnosis of MDS, 15/18 (83%) with the pre-marrow diagnosis of leukemia and 13/28 (46%) in those with the pre-marrow diagnosis of rule out lymphoma. Thirteen cases (18%) with possible MDS had post-bone marrow diagnoses of leukemia, anemia of chronic disease, myelofibrosis or medication-related changes. Five out of twenty two cases (23%) for anemia and 3/11 cases (27%) for thrombocytopenia without otherwise specified pre-bone marrow etiology had uncertain diagnosis after bone marrow biopsy. Conclusion: In about a fifth of patients necessitating a bone marrow, the diagnosis is discordant and can be surprising. It is also worth reporting that in these discordant results, non-hematological causes such as medications, anemia due to chronic diseases or conditions such as cirrhosis or splenomegaly from other etiologies were among the final diagnoses. Interestingly, 20% of the patients with unspecified pre-bone marrow diagnoses such as anemia or thrombocytopenia in our study had an unclear post-bone marrow diagnosis despite undergoing bone marrow biopsy. Our findings are a reminder that the bone marrow exam does not always lead to a definitive diagnosis and the need by exclusion to include in the differential non-hematological etiologies such as nutritional deficiencies, chronic kidney disease or autoimmune disorders. Disclosures No relevant conflicts of interest to declare.

Bortezomib In Combination with Dexamethasone and Liposome- Adriamycin Chemotherapy Regimen for Relapsed and Refractory Hodgkin Lymphoma: A Case Report

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4829-4829
Author(s):  
Yi Li ◽  
Gaofeng Zheng ◽  
Xiaoyan Han ◽  
Jimin Shi ◽  
Jingsong He ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hodgkin Lymphoma ◽  
Radiation Treatment ◽  
Conflicts Of Interest ◽  
Single Agent ◽  
Bone Marrow Aspiration ◽  
Salvage Chemotherapy ◽  
New Combination ◽  
Dependent Manner ◽  
Line Therapy

Abstract Abstract 4829 Bortezomib is effective in multiple myeloma, but also in other malignancies, particularly in lymphoma. Here we present a-23-year-old-male patient who complained of a progressive chest pain for 2 years. The CT scan of local hospital showed a mass that measured 5×14cm in the anterior mediastinum. He also had multiple lymphadenopathies in other areas. Tissue biopsy verified Classical Hodgkin lymphoma, nodular sclerosis (NDHL) IIIA. Then he had 12 cycles of ABVD, 1 cycle of MINE and CHOPE, 2 cycles of Hyper-CVAD regimen as prior treatments. Initial therapies induced good responses, but his disease progressed before he was transferred to hospital on December 29, 2009. His blood counts showed that WBC 30×10E9/L, with 89% neutrophils, Hb 99g/L, platelet 452 ×10E9/L. Bone marrow aspiration with immunophenotyping, analysis of TCR/IgH and the chromosome were normal. Biopsy reconfirmed the diagnosis of Hodgkin lymphoma. Lymphoma masses in his right neck, and severe edema with his right arm. He was treated with 2 cycle of IGVP regimen (ifosfamide, gemzar, vindesine, prednsone) and 2 cycles of GIP regimen (ifosfamide, gemzar, prednsone). With more progression, he subsequently received methotrexate and Ara-c with minimal response. Two weeks later, CT showed lung infection and pleural effusion, multiple lymphadenopathy. Lymph nodes decreased mildly and edema disappeared temporary after 3 cycles of R-CHOP. He was then treated with Bortezomib, dexamethasone and L-ADM. Four days after the first cycle, lymphoma masses decreased and significantly and his edema resolved. Due to the severe periphery neuropathy, he declined bortezomib, so 2 weeks later, the symptoms back again, and the FMD regimen (fludarabine, mitoxantrone, dexamethasone) was given. He then chose palliative radiation treatment and eventually died of pneumonia. Nearly 30%-40% of Hodgkin lymphoma relapsed after first line therapy even responds well at the initial stage. To these relapsed ones, the second line therapy including salvage chemotherapy and ASCT (autologous stem cell transplantation) act as the major treatment, but only a minority patients benefit from it. Treatment is limited to relapse and refractory ones hence they deserved more focus. The Bortezomib + L-ADM regimen exhibiting its efficacious after two cycles and the condition changed obviously. Our case is showing more antitumor activity of the new combination relative to either single agent alone. The new regimen responded well and with milder bone marrow suppression. Further study is still needed to demonstrate the relationship between the effective duration and treatment cycles. Some results showed that bortezomib inhibited cell proliferation and induced apoptosis in HL cell lines in a dose-dependent manner. At the same time, it is imperative to be aware of the adverse effects, such as peripheral neuropathy, thrombocytopenia and herpes zoster. As the patient mentioned above, the new regimen may work more optimally if more cycles were carried out, and he would have eventually responded if the complication was well controlled. Disclosures: No relevant conflicts of interest to declare.

Disseminated Tuberculosis with Hemophagocytic Lymphohistiocytosis (HLH)

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4686-4686
Author(s):  
Suparna Ajit Rao ◽  
Tarun K. Dutta ◽  
Rakesh V Naik ◽  
Aishwarya Krishnamurthy ◽  
Vinod K. Viswanath
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Leucocyte Count ◽  
Conflicts Of Interest ◽  
Total Leucocyte Count ◽  
High Grade Fever ◽  
Collagen Diseases ◽  
Disseminated Tuberculosis ◽  
Erythroid Hyperplasia

Abstract Abstract 4686 A 38 year old woman presented with high grade fever and jaundice for one month. Patient also had reduced appetite and loss of weight for the same period. On examination, patient had significant pallor, icterus and pedal edema. Ultrasonogram showed enlarged liver (20 cms) and enlarged spleen (17.2 cms). Patient was empirically treated for malaria. Her subsequent investigations revealed Hb 35g/dl, total leucocyte count 2×109/l, differential leucocyte count - neutro 82%, lympho 18%, platelet count − 59×109/l, and red cell indices were as follows: MCV 71.6 fL, MCH 21.6 pg/cell, MCHC 30.2 g/dl. Her reticulocyte count was 0.5%. Peripheral blood smear showed pancytopenia with moderate anisopoikilocytosis. Her total bilirubin was 4.2 mg/dl and serum ferritin was found to be 1720 μg/L. In view of pancytopenia and non-response to antimalarials, patient was treated in line of septicemia with piperacillin and tazobactam, and simultaneously a bone marrow biopsy was performed. Bone marrow biopsy subsequently revealed a hypercellular marrow with erythroid hyperplasia. Number of macrophages was increased with some showing ingested red cells (hemophagocytosis) within them. In view of fever, splenomegaly, pancytopenia, hemophagocytosis and hyperferritinemia, a diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made as per HLH 2004 diagnostic criteria. Bone marrow also revealed multifocal epithelial granulomas with caseation, pointing the etiology to that of disseminated tuberculosis. Patient expired before any anti-tuberculous treatment could be instituted. Causes of HLH are broadly malignancy, collagen diseases and infections. Though malignancy and collagen diseases are common causes in the Western countries, tuberculosis is an important cause in a tropical country like India. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Significant Variability in the Initial Workup of Thrombocytopenia in the Hematology Clinic - What Is the "Optimal" Workup?

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5966-5966
Author(s):  
Aishwarya Ravindran ◽  
Ronald S. Go ◽  
Kaaren K. Reichard ◽  
Ariela L. Marshall
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Large Scale ◽  
Hepatitis A Virus ◽  
Conflicts Of Interest ◽  
Medical Condition ◽  
Care Center ◽  
Laboratory Finding ◽  
Tertiary Care ◽  
Target Cells

Abstract BACKGROUND: Thrombocytopenia is a common hematologic condition associated with multiple etiologies ranging from benign to malignant to potentially life-threatening disorders. Given the heterogeneity of clinical presentations, available clinical information, and pertinent clinical history, there are inter-physician variations in the approach to the workup of thrombocytopenia in the hematology clinic. While a limited test repertoire may be adequate for many cases, more extensive testing may be warranted in others. We were interested in analyzing the various tests performed and testing approaches in the initial workup of thrombocytopenia. METHODS: We reviewed the records of 69 patients who were referred to our center between 2010 and 2015 for an initial workup of thrombocytopenia. We collected epidemiologic data, laboratory testing results, and pathologic findings. Pathologic results were classified as "normal" or "abnormal" and further subcategorized on the basis of review by two clinicians. Quantitative data were analyzed using JMP Pro 10.0.2 software. RESULTS: At the time of thrombocytopenia diagnosis, the median age was 59 years (range: 17-90) and majority were males (65%). The median platelet count was 91,000/µL (range: 3,000-146,000). Isolated thrombocytopenia was present in 51 cases (74%). Forty-four patients (64%) had a peripheral blood smear review and 4 (9%) contained abnormalities including hypogranular neutrophils, rouleaux formation, and target cells. Autoimmune workup included anti-platelet antibody (APA) in 34 (49%), anti-nuclear antibody (ANA) in 21 (30%), lupus anticoagulant (LA) in 4 (6%) and rheumatoid factor (RF) in 13 (19%) of cases. Autoimmune testing was positive for APA in 2 (5.9%), ANA in 4 (19%), LA in 0 (0%), and RF in 1 (8%) of patients who underwent testing, respectively. Common infectious workups included human immunodeficiency virus in 23 (33%), hepatitis A virus in 2 (3%), hepatitis B virus in 11 (16%), hepatitis C virus in 22 (32%), Epstein-Barr virus in 5 (EBV, 7%), cytomegalovirus in 7 (10%) and Helicobacter pylori in 5 (7%) of patients, and were negative in all cases except for one patient with evidence of active EBV infection. Sixteen patients (23%) underwent bone marrow biopsy, and 2 (12.5%) were diagnosed with hematologic malignancies including myelodysplastic syndrome and hairy cell leukemia. Based on results of these tests, 28 (41%) patients were diagnosed with primary immune thrombocytopenia, 19 (27%) with thrombocytopenia secondary to another medical condition, and 22 (32%) with thrombocytopenia of undefined etiology. CONCLUSION: Thrombocytopenia is a common laboratory finding, and workup involves significant inter-clinician variation, often involving multiple laboratory tests and in some cases invasive tests such as bone marrow biopsy. We found that autoimmune causes of thrombocytopenia were moderately common and infectious and malignant causes were rare. Our findings were based on a small cohort of patients but are likely to be representative of the clinical practice in a large tertiary care center. Large scale studies may be warranted to devise a protocol for a thorough yet cost-effective and stepwise initial workup of thrombocytopenia and to minimize unwarranted inter-clinician variation in such investigations. Disclosures No relevant conflicts of interest to declare.

scholarly journals Philadelphia Translocation in MDS: A Case Report and a Brief Review of the Literature Looking at Its Prevalence, Disease Progression, and Treatment Options

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Lakshmi Ramya Chelapareddy ◽  
Sandeep Sen
Keyword(s):  
Bone Marrow ◽  
Case Report ◽  
Kinase Inhibitors ◽  
Treatment Guidelines ◽  
Treatment Options ◽  
Germline Mutations ◽  
Definitive Treatment ◽  
Review Of The Literature ◽  
Molecular Abnormalities ◽  
Risk Of Progression

Myelodysplastic syndrome (MDS) is a group of clonal disorders characterized by ineffective and dysplastic hematopoiesis in the bone marrow with variable risk of progression to leukemia. MDS is characterized by specific karyotypic and molecular abnormalities. The t(9 : 22) Philadelphia translocation is not a common abnormality found in MDS, and it is not included in the prognostic indices for germline mutations. There are no definitive treatment guidelines for these patients either. Here, we reviewed previously reported cases of MDS with the Philadelphia translocation with a goal to determine their prognosis and treatment options, specifically the tyrosine kinase inhibitors (TKIs).

Uptake of New Therapeutics in the Treatment of Multiple Myeloma (MM) in Germany (GER) - Results from a Representative Multicentre Treatment Survey 2006.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5110-5110
Author(s):  
Ralf Angermund ◽  
W. Knauf ◽  
M. Freund ◽  
M. Nowrousian ◽  
H. Einsele ◽  
...  
Keyword(s):  
Treatment Guidelines ◽  
Treatment Options ◽  
High Dose Chemotherapy ◽  
Minor Role ◽  
High Dose ◽  
Primary Therapy ◽  
University Hospitals ◽  
Treatment Standard ◽  
Line Therapy ◽  
A Minor

Abstract Background: Over the past decade new treatment options and drugs significantly altered the treatment paradigm and treatment guidelines for patients with MM. We reported in 2005 initial results from a treatment survey in GER (Freund ASH2005# 5158; Angermund, ASH2005, #5156). Bortezomib (Velcade®, Vel) at that time (6 months (m) after approval) was the 2nd most frequently (freq) used drug after dexamethasone (dex) in the approved indication (3rd line) and played a minor role in 2nd line therapy. The survey performed in 1st quarter 2006 (1.5 years (y) after the initial survey), used identical methods and questionnaires. These results allow for monitoring of changes in treatment patterns. Methods: The method of this representative analysis was provided elsewhere (Freund ASH2005# 5158). The data presented here are a subset analysis based on 66 sites and 428 patients. Results: 230 male and 195 female patients, 43% at the time of analysis for decision on primary therapy, 27% for secondary treatment and 30% for further treatment were included in this analysis. The centers participating were 11% university hospitals (UH), 38% non-university hospitals with specialized (SH) and 20% without specialized (NSH) haematology department, and 32% office-based haematologists (OBH). The main treatment in primary therapy did not change (table) whereas Vel gets more commonly used. The increase in Vel in all treatment lines is mainly due to SH (64 (2006) vs. 52 % (2004); OBH: 32 (2006) vs. 32% (2004); NSH: 0 (2006) vs. 2 % (2004); UH 4 (2006) vs 14% (2004)). In 2nd line Vel was the 2nd most prescribed drug (31%) (1st dex 41%; 2004: Vel 8% ranked 10th) mainly in UH/SH in patients below 60 y and after primary high dose chemotherapy (HDCT) (35% Vel vs. 28 % all) and with longer remission period (median 9 m Vel, median 7 m all). Within treatment ≥ 3rd line, Vel was the most freq used drug followed by dex (42% and 38% respectively). For patients currently in 1st remission Vel was most freq planned as next therapy (45%) followed by lenalidomide (len) (19%) and dex (12%). Vel was mostly used by SH following melphalan or VAD/VID treatment (multivariate analysis). Len and thalidomide (thal) (both drugs not approved in GER at that time) are rarely used in MM therapy (0% len vs. 10% thal in ≥ 2nd line). Conclusion: New approved treatment options like Vel are quickly integrated into treatment behaviour in GER, mainly used in SH within approved indication, whereas not approved drugs like len or thal play only a minor role. 9 m after approval for 2nd line therapy, Vel increased uptake within ≥ 2nd line indicating this drug’s possibility to become a future treatment standard in pretreated patients. In order to detect the dynamic of change of treatment of MM, further follow up is planned.

Baseline Staging Evaluation in Lymphoma: The Role of FDG PET, CT, and Bone Marrow Biopsy

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2640-2640
Author(s):  
Andrew D Zelenetz ◽  
Jocelyn Maragulia ◽  
Steven M. Horwitz
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Fdg Pet ◽  
Conflicts Of Interest ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Contrast Enhanced Ct ◽  
Incident Cases ◽  
The Impact ◽  
Fdg Avidity

Abstract Abstract 2640 BACKGROUND: The revised response criteria for malignant lymphoma (Cheson et al JCO 25:579 2007) incorporated FDG PET for determination of response. This was strongly recommended for patients with curable lymphomas (diffuse large B-cell lymphoma [DLBCL] and Hodgkin lymphoma [HL]). For incurable lymphomas PET was not recommended unless response was a major trial endpoint. Part of the reservation regarding the use of PET for response evaluation in incurable lymphomas was based on the potential variability in FDG avidity at baseline. This retrospective review was performed to understand the frequency of FDG avidity across a range of lymphoma histologies and compare diagnostic yield to CT. Furthermore, diagnostic utility of bone marrow biopsy was evaluated in patents with DLBCL and HL compared to identification of disease by FDG PET and CT scan. METHODS: After obtaining a waiver of authorization from the MSKCC Institutional Review Board patients the DAVInCI data mining tool was used to retrospectively identify patients with the diagnosis of lymphoma who had both a FDG-PET scan and CT at diagnosis available in the PACS. Either the FDG-PET or the CT had to be informative (which excluded patient with CS I resected disease). The sites of disease were recorded for PET (including SUVs) and for CT. The impact on the CS at diagnosis was determined. DLBCL and HL patients with bone (B) or bone marrow (BM) involvement were identified by being positive on any one modality: BM biopsy; FDG-PET; or CT. RESULTS: Data, including imaging, from 522 incident cases of lymphoma were reviewed. FDG PET performed for lymphoma at initial diagnosis demonstrated FDG-avid disease in 97.3% (508/522) of cases; there was some variability across histologies (Table 1). There was a strong correlation between CT and PET. PET identified more disease in 0–32.3% of cases depending on histology and CT was more informative in 3.2–33.3% of cases. CT tended to be more informative where the median SUV of PET was relatively low (SLL, MZL, MCL). The impact of FDG PET on Ann Arbor stage was modest but was greatest for SLL and T-cell lymphoma (Table 1, Change Stage). Identification of bone (B) and bone marrow (BM) disease in HL and DLBCL was examined. 57 patients with DLBCL were found to have B/BM involvement by BM, CT, or PET. PET was the most sensitive test for B/BM disease (50/57 88%). CT identified bone disease in 33/37 (58%) of cases but these were strictly a subset of the PET positive cases. However, BM biopsy identified involvement in 7 (15.2%) cases which were negative by PET. For HL, 20 patients were found to have B/BM disease. FDG PET identified all 20 cases, CT 12 cases and BM biopsy only 3. CONCLUSIONS: FDG-PET is positive in baseline in 97% of cases of lymphoma across all histologies. However, there is discordance between CT and PET in 28% of cases. Therefore, for clinical trials baseline contrast enhanced CT and FDG-PET are both necessary at baseline to full identify sites of disease. Outside the setting of a clinical trial, clinical discretion should be used in choosing the appropriate pre-treatment imaging. In the case of B/BM disease FDG-PET was the most informative modality in DLBCL and HL. However, bone marrow biopsy remains an essential part of the diagnostic evaluation of DLBCL since 7/20 bone marrow positive cases were not identified on FDG-PET or CT. The utility of the bone marrow biopsy in HL was questionable as FDG PET identified all cases of B/BM disease. Disclosures: No relevant conflicts of interest to declare.

Bone Marrow Biopsy May Be Required During the Initial Evaluation of Individuals with Asymptomatic Monoclonal Gammopathy

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5067-5067
Author(s):  
Meletios Athanasios Dimopoulos ◽  
Evangelos Terpos ◽  
Maria Gkotzamanidou ◽  
Evangelos Eleutherakis-Papaiakovou ◽  
Magdalini Migkou ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Plasma Cell ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
Conflicts Of Interest ◽  
Incidental Finding ◽  
M Protein ◽  
Monoclonal Protein

Abstract Abstract 5067 The incidental finding of a monoclonal gammopathy during workup for various conditions or in the context of a routine check-up is increasingly common. Several “patients” are then referred for diagnostic evaluation of their monoclonal gammopathy and additional workup is needed. It has been proposed that a bone marrow (BM) aspirate and biopsy is indicated when the monoclonal protein (M-protein) is ≥1.5 g/dL, when abnormalities are noted in the complete blood cell count, serum creatinine level, serum calcium level, or radiographic bone survey, in individuals with non-IgG monoclonal gammopathy and in those with an abnormal serum free light chain (FLC) ratio. The aim of this study was to identify factors that could aid in the evaluation of individuals presenting with asymptomatic monoclonal gammopathy and in whom invasive diagnostic testing with a bone marrow biopsy is considered. Thus, we analyzed our database and identified patients who were referred to the Department of Clinical Therapeutics of the University of Athens, Greece, for evaluation of asymptomatic monoclonal gammopathy and in whom a BM trephine biopsy, a serum and urine protein electrophoresis (SPEP) with immunofixation and quantitative immunoglobulins were performed. SPEPs were scanned and M-protein was measured using imaging analysis software. Patients with a monoclonal M-protein ≥ 3 g/dl (30 g/L), i.e. those diagnosed with asymptomatic/smoldering myeloma (SMM) or Waldenstrom's macorglobulinemia based on the standard criteria, were not included in the analysis. Clonality of BM plasma cells or lymphoplasmacytes was assessed by immunohistochemistry. Patients who eventually were diagnosed with plasma cell related conditions (i.e. amyloidosis, peripheral neuropathy, dermatoses, etc.) were also excluded from the analysis. Our analysis included 161 patients: 53% were females, median age was 64 year (range 33–89 years), 53% had a monoclonal IgG protein, 15.5% had a monoclonal IgA protein, 24% a monoclonal IgM protein and 2.5% had only a monoclonal light chain, while 4% had a biclonal protein. In 64% of patients the monoclonal light chain was kappa and in 37% was lambda. The median serum M-protein was 0.948 g/dl (range 0.1–2.99 g/dl); 52% of patients had an M-protein of <1 g/dl and 79% of <2 g/dl. Immunoparesis of at least one of the uninvolved immunoglobulins was present in 38% of cases and of both of the uninvolved immunoglobulins in 6%. Median BM infiltration by monoclonal plasma cells or lymphoplasmacytes was 15%. In 66.5% of individuals there was a BM infiltration of ≥10% by monoclonal plasma cells or lymphoplasmacytes, while in 10% of the studied cases the BM infiltration was ≥50%. A significant correlation of the size of M-protein and of the infiltration of the BM was found (R=0.592, p<0.001). However, 27% of patients with M-protein <0.5 g/dl had ≥10% clonal plasma cells or lymphoplasmacytes in their BM biopsies. The respective rates were 46% for those with M-protein <1 g/dl, 54% for those with M-protein 1.5 g/dl and 58% for those with M-protein <2 g/dl. Ninety per cent of those who had immunoparesis of at least one of the uninvolved immunoglobulins had ≥10% clonal plasma cells or lymphoplasmacytes. A BM infiltration of ≥10% was more frequent in individuals with a monoclonal IgG or IgA protein (72% and 80%, respectively) vs. 45% of those with a monoclonal IgM protein (p=0.015). Light chain isotype, age and gender were not predictive of the degree of BM plasma cell infiltration. In multivariate analysis, immunoparesis of at least one of the uninvolved immunoglobulins (OR: 6.45, 95% CI: 2.32–18, p<0.001), an IgG or IgA monoclonal protein (OR: 2.67, 95% CI: 1.1–6.4, p=0.028) and an M-protein of ≥1 g/dl (OR: 5.4, 95% CI: 2.23–13) were independently associated with the presence of ≥10% of clonal infiltration in BM biopsy. By combining the above risk factors we found that in those who had all three, 97% had ≥10% clonal cells in the BM biopsy, while in those with 0–1 of the above factors the probability to find ≥10% clonal cells was 43%. These findings indicate that even patients with low risk for BM infiltration by clonal plasma cells, may be diagnosed as SMM when a BM biopsy is performed. In conclusion, our data on a large number of individuals with asymptomatic monoclonal gammopathy who underwent a BM biopsy may indicate that the latter exam may provide useful information and could be included in the standard initial workup of these individuals. Disclosures: No relevant conflicts of interest to declare.

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