Abstract #804110: Recognizing A Rare Disease: Primary Thyroid Diffuse Large B Cell Lymphoma

2020 ◽  
Vol 26 ◽  
pp. 264-265
Author(s):  
Jessica Kunadia
Keyword(s):  
B Cell ◽  
Rare Disease ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

VP46 German Claims Data In Rare Disease HTA: Diffuse Large B-cell Lymphoma

2019 ◽  
Vol 35 (S1) ◽  
pp. 87-87
Author(s):  
Aljoscha Neubauer ◽  
Susanne Guthoff-Hagen ◽  
Jacob Menzler ◽  
Carsten Schwenke ◽  
Markus Rueckert ◽  
...  
Keyword(s):  
B Cell ◽  
Rare Disease ◽  
Claims Data ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Real World Data ◽  
Oncological Treatment ◽  
Large B Cell Lymphoma ◽  
Sick Fund ◽  
Large B Cell

IntroductionIn rare disease areas representative data are scarce. Routine sick fund claims data provide a meaningful and reliable base for the in- and outpatient treatment landscape. This real-world data (RWE) from Germany was used to describe treatment patterns for Diffuse Large B-cell Lymphoma (DLBCL), the most frequent and aggressive non-Hodgkin lymphoma type in adults.MethodsClaims data from several sick funds of 4.8 Million insured were analyzed. Diagnosis of non-follicular Lymphoma (C83) was confirmed in 2.178 patients, DLBCL (C83.3) in 819 patients. The analysis was age- and gender-adjusted, observational period was 2014 and 2015. Treatments were analyzed for hospitalization and medication based on ATC-Code, Pharma Central Number and coded diagnoses (per ICD).ResultsMean age of DLBCL patients was 60.3 years, with two peaks at 50-54 and 70-74 years. Total costs for patients with DLBCL averaged 25.048 EUR versus 1.259 EUR in healthy insured. Charlson comorbidity index (CCI) of 4.58 indicates clinical relevance and severity. Comorbidities included several psychiatric diagnoses such as depression in every fifth patient. Mean 3.2 hospitalizations with average 31.5 hospital days were observed in DLBCL patients. Forty-seven percent of patients during observational time-frame did not receive oncological treatment, including relapsed / refractory patients. Only few patients received stem cell transplantation (2.6 percent) or radiation (3.9 percent). Most pharmacological treatments were Rituximab (RTX) + CHOP (57 percent), followed by RTX mono therapy (25 percent) or RTX in combination with Bendamustine (8 percent).ConclusionsDespite limitations in sick fund claims analyses, these provide a reasonable database for rare diseases. They allow standard treatment pathway- and longitudinal analyses. All DLBCL patients frequently required hospitalization and generated significant costs. A high unmet medical need exists for treatments other than palliative care, especially for a tolerable and effective outpatient therapy in elderly relapsed / refractory DLBCL.

scholarly journals T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Uroosa Ibrahim ◽  
Gwenalyn Garcia ◽  
Amina Saqib ◽  
Shafinaz Hussein ◽  
Qun Dai
Keyword(s):  
T Cell ◽  
B Cells ◽  
B Cell ◽  
Rare Disease ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Biologically Active ◽  
Large B Cell Lymphoma ◽  
Large B Cell

T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkin’s lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy. On presentation, he had a fever of 105°F. Laboratory work-up was consistent with pancytopenia, elevated lactate dehydrogenase, elevated D-dimer, and a ferritin of 24,247 ng/mL. The patient was started on steroid therapy. An excisional biopsy of the right inguinal lymph node was consistent with a diagnosis of THRLBCL and the patient subsequently received six cycles of chemotherapy with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) after which a PET-CT scan showed no evidence of biologically active disease and ferritin was down to 822 ng/mL. We discuss the clinical manifestations and diagnostic and therapeutic considerations of this rare disease along with a review of reported cases in the literature.

scholarly journals Ileo-Colonic Fistula Due to Diffuse Large B Cell Lymphoma: Unusual Presentation of a Rare Disease

Cureus ◽  
2021 ◽  
Author(s):  
Godson Senyondo ◽  
Saif Bella ◽  
Atif Saleem ◽  
Sarah A Mehdi ◽  
Jagmohan Sidhu
Keyword(s):  
B Cell ◽  
Rare Disease ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Unusual Presentation ◽  
Colonic Fistula ◽  
Large B Cell Lymphoma ◽  
Large B Cell

scholarly journals Acute Kidney Injury as Presenting Symptom of Primary Lymphoma of the Uterus: Uncommon Presentation of a Rare Disease

2021 ◽  
Author(s):  
Alexandra Snyder ◽  
Ida Dhanuka ◽  
Haiyan Li ◽  
Fouzia Shakil ◽  
Liying Han ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
B Cell ◽  
Rare Disease ◽  
Cell Lymphoma ◽  
Kidney Injury ◽  
B Cell Lymphoma ◽  
Diagnosis And Treatment ◽  
Bilateral Hydronephrosis ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Abstract Background: Primary uterine lymphoma is a rare disease, with diffuse large B cell lymphoma being the most common subtype. There are a limited number of reports, series, and reviews in the literature on this disease and its variable clinical presentations. Further data is needed to prevent delay in diagnosis and treatment.Case: We present the case of a 73-year-old with acute kidney injury secondary to severe bilateral hydronephrosis in setting of an enlarged uterus, confirmed to be primary uterine diffuse large B cell lymphoma.Conclusion: Acute kidney injury secondary to severe bilateral hydronephrosis is an uncommon clinical presentation of primary uterine lymphoma. This case highlights the importance of heightened awareness of such rare presentations so not to delay diagnosis and treatment of this disease, and emphasizes benefit of surgical resection in the diagnosis and treatment of this disease.

scholarly journals Hypercalcemia in T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: An Unusual Presentation of a Rare Disease and Literature Review

2019 ◽  
Vol 10 (6) ◽  
pp. 231-236
Author(s):  
Gabriella A. Conte ◽  
Jonathan S. Harmon ◽  
Marjolein L. Le ◽  
Xiu Sun ◽  
Jake W. Schuler ◽  
...  
Keyword(s):  
T Cell ◽  
Literature Review ◽  
B Cell ◽  
Rare Disease ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Unusual Presentation ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Lymphome

Praxis ◽  
2016 ◽  
Vol 105 (1) ◽  
pp. 47-52 ◽  
Author(s):  
Andreas Lohri
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Staging System ◽  
Maligne Lymphome ◽  
Interim Pet ◽  
Large B Cell Lymphoma ◽  
Ann Arbor ◽  
Large B Cell

Zusammenfassung. Maligne Lymphome unterteilen sich zwar in über 60 Entitäten, das grosszellige B-Zell-Lymphom, das follikuläre Lymphom, der Hodgkin und das Mantelzell-Lymphom machen aber mehr als die Hälfte aller Lymphome aus. Im revidierten Ann Arbor staging system gelten die Suffixe «A» und «B» nur noch für den Hodgkin. «E» erscheint nur noch bei Stadien I und II. Eine Knochenmarksuntersuchung wird beim Hodgkin nicht mehr verlangt, beim DLBCL (Diffuse large B cell lymphoma) nur, falls das PET keinen Knochenmark-Befall zeigt. Der PET-Untersuchung, speziell dem Interim-PET, kommt eine entscheidende Bedeutung zu. PET-gesteuerte Therapien führen zu weniger Toxizität. Gezielt wirkende Medikamente mit eindrücklicher Wirksamkeit wurden neu zugelassen. Deren Kosten sind hoch. Eine strahlen- und chemotherapiefreie Behandlung maligner Lymphome wird in Zukunft möglich sein.

Sign in / Sign up

Export Citation Format

Share Document