Cutaneous B-cell Research Articles

Qol-O-06 - Development of EORTC/CLTF questionnaire to assess HRQOL in primary cutaneous T-cell and B-cell lymphomas: phase I

<h3>Background:</h3> Health-related quality of life (HRQoL) in patients with cutaneous lymphoma (CL) is negatively impacted by a large burden of disease symptoms including intractable itch, pain, discomfort and socially stigmatising cosmetic disfigurement which may adversely affect patient self-esteem and interpersonal relationships. There is currently no cure for CL and as such, accurate determination of patient HRQoL is of paramount importance. To date, the impact of both skin disease and of a cancer diagnosis on patient HRQoL in CL is assessed using separate, non-CL-specific questionnaires. A composite HRQoL questionnaire addressing all aspects of the disease is lacking. <h3>Methods:</h3> The EORTC Quality of Life Group questionnaire development guidelines were followed. For phase I, semi-structured interviews were conducted in 15 patients with early-stage mycosis fungoides (IA–IIA), 30 patients with late-stage mycosis fungoides/Sézary syndrome (IIB–IVB) (15 patients <3 years and >3 years from diagnosis) and 15 patients with cutaneous B cell lymphoma. Collaborators communicated during the patient recruitment to ensure balanced patient selection with respect to age, gender, stage, duration of the disease, and number of (previous) treatments. <h3>Results:</h3> Patients were recruited in 5 countries (6 centres) across Europe. So far, 62 patients participated (target 60) from each of the following representative patient groups: early MF (stage IA–IIA), late MF (stage IIB–IVB) and cutaneous B cell lymphoma. Data from patient interviews and questionnaires is being analyzed using basic quantitative analyses, including generation of descriptive statistics (e.g., %missing data, means and standard deviations, floor and ceiling effects, etc.), prevalence (number of patients who experienced each complaint i.e. who scored 2, 3 or 4, divided by the total number of patients who completed that item, multiplied by 100), priority ratings (number of patients and professionals who have given priority ratings to each item) and range of responses for each item. The data from the debriefing questions will be reported both quantitatively and qualitatively (in narrative form). <h3>Conclusion:</h3> Once completed, phase I issues will be operationalised into items and any new items created in a style and format compatible with the EORTC quality of life group translation guidelines (phase II). The preliminary module will be pre-tested in phase III followed by large-scale international field-testing in phase IV. A comprehensive HRQoL questionnaire (module) for patients with CL is being developed to more accurately measure assessment of the impact of HRQoL in patients with skin lymphomas.

Genomic Landscape of Primary Cutaneous Diffuse Large B Cell Lymphoma, Leg Type

Primary cutaneous diffuse large B-cell lymphoma, leg type (DLBCL-LT) is an aggressive subtype of cutaneous B cell lymphoma (CBCL) with a poor prognosis. It is most often diagnosed on the legs of the elderly, frequently relapses, and has a tendency towards extracutaneous dissemination. Current treatment strategies are ineffective and revolve around surgical excision, multiagent chemotherapy (e.g. R-CHOP) and radiation therapy, with estimated 5-year survival rates of ~50%. Identifying genetic mutations and altered pathways specific for DLBCL-LT is crucial for the diagnosis and development of effective targeted therapies for this condition.We performed whole exome capture and massively parallel sequencing on 13 tumor samples of DLBCL-LT (4 matched and 9 unmatched) and 5 samples of cutaneous diffuse large B cell lymphoma, not otherwise specified (DLBCL-NOS) (1 matched and 4 unmatched). This is the largest whole exome cohort of cutaneous DLBCL to date. We applied algorithms routinely used by our lab to identify putative cancer promoting mutations and to compare the genetic landscape with DLBCL, both nodal and extranodal.Broadly, the landscape of mutations in DLBCL-LT is similar to that of activated B-cell like (ABC) DLBCL and most analogous to primary central nervous system lymphoma (PCNSL), a type of extranodal ABC DLBCL. Similar to PCNSL, we found frequent disease-defining oncogenic gene mutations in MYD88 (85%), CD79B (38%), and TBL1XR1 (38%), and loss of function mutations in tumor suppressor genes KMT2D (15%), CDKN1B (15%), and CD58 (8%). We observed frequent mutations in PIM1 (54%), OSBPL10 (31%), BTG2 (31%), and IGLL5 (15%), which are either known or proposed targets of activation-induced cytidine deaminase (AID) and aberrant somatic hypermutation (aSHM) also seen in PCNSL. Importantly and similarly to PCNSL, we found alterations at the PDL-1 and PDL-2 locus, but instead of copy number gains, we discovered frequent PD-L1 and PD-L2 translocations (40%) that may translate into overexpression of PD-L1 .This spectrum of mutations differed significantly compared to our DLBCL-NOS cohort, which did not have the above oncogenic mutations and lacked any PD-L1 or PD-L2 chromosomal alterations. Finally, we report novel oncogenic mutations in DLBCL-LT including MYD88 p.S243N, CD79B p.L199P, CCND3 p.T283P and p.P284S, BRAF p.V600E, and CARD11 p.R113Q and novel loss of function mutations in KMT2D, CDKN1B, CD58, MSH2, PIK3R1, MSH2, and BMF .Collectively, we identified recurrent mutations in multiple key cancer-related biological pathways, including epigenetic modulators (TBL1XR1, HIST1H1E, and KMT2D), NF-κB signaling pathway (MYD88, CD79B, and CARD11), MAPK signaling pathway (BRAF), PI3K signaling pathway (PIK3R1), JAK/STAT signaling pathway (PIM1), cell cycle regulators (CCND3, CDKN1B and BTG2), and immune escape (CD58). All of the DLBCL-LT samples in our cohort contained one or more gene mutations that can be targeted by agents that are currently FDA-approved or under clinical trials, such as BRAF inhibitors, JAK/STAT inhibitors, PIM inhibitors, PI3K inhibitors, NF-kB inhibitors, MYD88 inhibitors, and PD-1/PDL-1 inhibitors.Altogether, our data identified targetable putative driver gene mutations in DLBCL-LT. These mutational signatures bear broad similarities to PCNSL. Because of the relative rarity of this disease, we hope these efforts will allow for its inclusion, when relevant, in trials involving genotypically similar DLBCL. DisclosuresNo relevant conflicts of interest to declare.

Helicobacter pylori and skin disorders: a comprehensive review of the available literature.

Helicobacter pylori is a Gram-negative bacterium identified for the first time about 30 years ago and commonly considered as the main pathogenic factor of gastritis and peptic ulcer. Since then, it was found to be associated with several gastrointestinal and extra-gastrointestinal diseases. Helicobacter pylori is also associated with many skin disorders including, but not limited to, chronic urticaria, rosacea, lichen planus, atopic dermatitis, psoriasis, pemphigus vulgaris, vitiligo, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, primary cutaneous marginal zone B-cell lymphomas and cutaneous T-cell pseudolymphoma. Literature up to September 2020 shows that clear evidence exists only for some of the mentioned associations, while in the majority of cases, data appear contrasting. The aim of this review is to summarize the available studies on the topic and draw possible conclusions. Further clinical and laboratory studies are needed to assess the real plausibility and relevance of these associations, as well as the possible role of Helicobacter pylori with the underlying pathogenic mechanisms.

A Phase 1 Study of HMPL-689, a Small Molecule, Highly Selective, and Potent Inhibitor of Phosphoinositide 3 Kinase-Delta, in Patients with Relapsed or Refractory Lymphoma

Background: The B-cell receptor (BCR) signaling pathway plays a critical role in the pathogenesis of lymphomas, particularly in non-Hodgkin lymphomas (NHL). Despite availability of therapeutic agents targeting BCR pathway, there is an unmet medical need for more effective and well-tolerated therapeutic agents for patients with advanced relapsed, refractory, or resistant NHL. HMPL-689 is a novel, orally available, highly selective, and potent small molecule inhibitor of phosphosinositide 3 kinase-delta (PI3Kδ), a crucial signaling transduction molecule in the BCR signaling pathway. A global clinical study of HMPL-689 is currently ongoing in the USA and the EU countries of France, Italy, Poland, and Spain. Preliminary results from dose escalation and expansion stages of this study are expected soon. Herein is the description of an ongoing phase 1, open-label, multi-center, single-arm study of HMPL-689 in patients with advanced relapsed, refractory, or resistant (R/R) NHL (NCT03786926). Study Population: Target patient population is adult patients with histologically confirmed advanced relapsed, refractory, or resistant NHL. To be eligible for enrollment, patients must have exhausted all approved therapeutic options available. Patients are ineligible for the study if they have primary central nervous system lymphoma. Objectives and Endpoints: The primary objective is to determine the maximum-tolerated dose (MTD)/recommended phase 2 dose (RP2D) of HMPL-689. The primary endpoints are the incidence of dose-limiting toxicity (DLT) and safety parameters, including treatment-emergent adverse events and laboratory abnormalities The secondary objective is to evaluate the pharmacokinetic (PK) parameters and preliminary efficacy. The secondary endpoints include concentration-time profiles, PK parameters, and efficacy parameters, including objective response rate, time to response, duration of response, and progression-free survival. Study Design: Study consists of a dose escalation and dose expansion stages. The dose-escalation stage utilizes a mTPI-2 design, with anticipated enrollment of approximately 24 patients until MTD is reached, and RP2D is determined. The proposed doses for escalation cohorts are 10, 15, 20, 25, 30, 35, 40, 45, and 50 mg, QD, PO in a 28-day cycle. Patients will be treated until disease progression, intolerable toxicity, no further benefit, withdrawal, end of study, or death. The expansion stage will be dosed at the MTD. Approximately 10 patients will be enrolled in each of the following cohorts: Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL),Mantle cell lymphoma (MCL),Follicular lymphoma (FL)Marginal zone lymphoma (MZL)Peripheral T-cell lymphoma (PTCL)Cutaneous B-cell lymphomaWaldenström's macroglobulinemia / lymphoplasmacytic lymphoma (WM) Statistical Methods: The maximum sample size in the dose-escalation stage will be determined based on the accumulated safety data and the mTPI-2 design. The maximum sample size under the mTPI-2 method is k × (d + 1), where k denotes the cohort size and d denotes the number of doses. The minimum cohort size in this study is 3 patients per cohort. To ensure that the highest dose (if needed) is reached, it is estimated that approximately 33-39 patients will be needed in this study. For dose expansion, approximately 70 patients (10 in each cohort) will provide robust safety data in the patient populations studied. For a given AE with a true rate of 10%, 5%, or 1%, the probability of observing at least one such AE in 70 patients is 99.9%, 97.2%, and 50.5%, respectively. For preliminary assessment of anti-tumor activity based on ORR, if at least 8 patients in a specific lymphoma subtype are evaluable for tumor response, the chance of observing at least one response is 94.2%, if the true ORR is 30%. Data will be summarized by dose level, subtype of malignancy, and overall as appropriate. Continuous assessments will be summarized by number of patients (n), mean, standard deviation, median, minimum and maximum. Significance: This study is the first global clinical study of HMPL-689, a novel inhibitor of the BCR signaling pathway, which is currently enrolling patients with advanced relapsed, refractory, or resistant NHL who have exhausted all approved therapeutics options available. Disclosures Lawrence: Hutchison MediPharma International, Inc:Current Employment, Current equity holder in publicly-traded company.Hahka-Kemppinen:Hutchison MediPharma International, Inc:Current Employment, Current equity holder in publicly-traded company.Cohen:Janssen, Adicet, Astra Zeneca, Genentech, Aptitude Health, Cellectar, Kite/Gilead, Loxo:Consultancy;Genentech, BMS, Novartis, LAM, BioInvent, LRF, ASH, Astra Zeneca, Seattle Genetics:Research Funding.Kania:Hutchison MediPharma International, Inc:Current Employment, Current equity holder in publicly-traded company.

A Phase 1 Study of HMPL-523, Highly Selective and Potent a Small Molecule Inhibitor of Spleen Tyrosine Kinase in Patients with Relapsed or Refractory Lymphoma

Background: The B-cell receptor (BCR) signaling pathway plays a critical role in the pathogenesis of lymphomas, particularly in non-Hodgkin lymphomas (NHL). Despite availability of therapeutic agents targeting BCR pathway, there continue to be unmet medical need for more effective and less toxic therapeutic agents for patients with advanced relapsed, refractory or resistant lymphoma. HMPL-523 is a novel, orally available, highly selective, and potent small molecule inhibitor of spleen tyrosine kinase (SYK), a crucial component of BCR signaling pathway. A clinical study is currently ongoing in the USA and in EU countries of France, Italy, Poland, and Spain. Preliminary results from dose escalation and expansion stages of these trials are expected soon. Herein is the description of an ongoing phase 1 open-label, multi-center, single-arm study of HMPL-523 in patients with advanced relapsed, refractory or resistant (R/R) NHL (NCT03779113). Study Population: Targeted patient population is adult patients with histologically confirmed advanced relapsed, R/R NHL. To be eligible for enrollment, patients must have exhausted all approved therapeutic options available. Objectives and Endpoints: The primary objective is to assess safety and tolerability of HMPL-523 and to determine the maximum-tolerated dose (MTD)/recommended phase 2 dose (RP2D). The primary endpoints are the incidence of dose-limiting toxicity (DLT) and safety parameters, including treatment-emergent adverse events and laboratory abnormalities The secondary objectives are to characterize the pharmacokinetic (PK) parameters and to evaluate the safety and preliminary efficacy of HMPL-523. The secondary endpoints are the incidence of treatment-emergent adverse events (TEAE), PK parameters, and efficacy parameters, including objective response rate, duration of response, time to response, and progression-free survival. Study Design: Study consists of a dose escalation stage and an expansion stage. The dose-escalation utilizes a modified 3+3 design, with anticipated enrollment of approximately 24 patients until MTD is reached, and RP2D is determined. The proposed doses of escalation cohorts are 100, 200, 400, 600 and 800 mg, QD, PO in a 28-day cycle. Patients will be treated until disease progression, intolerable toxicity, no further benefit, withdrawal, end of study, or death. The expansion stage will be dosed at the MTD to further evaluate safety, tolerability, PK, drug exposure, target engagement, PD and their correlation with clinical benefit of HMPL-523. Approximately 10 patients will be enrolled in each of the following cohorts: Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL),Mantle cell lymphoma (MCL),Follicular lymphoma (FL)Marginal zone lymphoma (MZL)Peripheral T-cell lymphoma (PTCL)Cutaneous B-cell lymphomaWaldenström's macroglobulinemia / lymphoplasmacytic lymphoma (WM) Statistical Methods: For the escalation stage, the sample size is based on the dose-escalation rules of the 3+3 design. For a given AE with a true rate of 10%, 5%, or 1%, the probability of observing at least one such AE in a given cohort of 6 patients is 46.9%, 26.5%, and 5.8%, respectively. For the dose expansion stage, a total of 70 patients (10 patients per expansion cohort) will provide robust safety data in the study patient populations. For a given AE with a true rate of 10%, 5%, or 1%, the probability of observing at least one such AE in 70 patients is 99.9%, 97.2%, and 50.5%, respectively. For preliminary assessment of anti-tumor activity based on ORR, if at least 8 patients in a specific lymphoma subtype are evaluable for tumor response, the chance of observing at least one response is 94.2%, if the true ORR is 30%. Safety parameters, including DLTs, recorded TEAEs, clinical laboratory parameters, vital signs, 12-lead ECG parameters and physical examination findings, will be summarized by dose level across both dose escalation and dose expansion stages. Efficacy endpoints will be summarized by dose level for each type of malignancy. Significance: This study is the first global clinical study of HMPL-523, a novel inhibitor of the BCR signaling pathway currently enrolling patients with advanced relapsed, refractory or resistant NHL who have exhausted all approved therapeutics options available. Disclosures Lawrence: Hutchison MediPharma International, Inc:Current Employment, Current equity holder in publicly-traded company.Hahka-Kemppinen:Hutchison MediPharma International, Inc:Current Employment, Current equity holder in publicly-traded company.Kania:Hutchison MediPharma International, Inc:Current Employment, Current equity holder in publicly-traded company.

伴显著浆细胞分化的原发性边缘区B细胞淋巴瘤一例

伴显著浆细胞分化的原发性边缘区B细胞淋巴瘤一例

Primary cutaneous diffuse large B-cell lymphoma, leg type – case report and literature review

Primary cutaneous diffuse large B-cell lymphoma, leg type – case report and literature review

Primary cutaneous diffuse large B-cell lymphoma, leg-type: The Mayo Clinic experience.

e20044 Background: Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg-type (PCDLBCL) is a rare lymphoma comprising 4% of all cutaneous lymphomas with an aggressive clinical course. We investigated disease characteristics and outcomes among patients PCDLBCL. Methods: Following IRB approval, we identified patients retrospectively with PCDLBCL treated at the Mayo Clinic Cancer Center 1998-2019. Results: 45 pts were identified with a median age of 71 years (Range- 48-95) and 28 (62%) were female. Disease locations were legs (62%), arms (22%), head (11%), trunk (2%) and multiple sites (2%). At diagnosis, (51%) had a single lesion, (29%) had multiple lesions in 1-2 contiguous regions, (16%) had multiple lesions in non-contiguous regions or &gt; 2 regions and data was not available (4%). Immunohistochemistry showed positive staining for BCL-2 (92%), BCL-6 (76%), c-MYC (62%), MUM1 (95%), CD10 (11%), and cytoplasmic IgM (8/9- 89%). By Han’s criteria, (95%) had activated B cell lymphoma (ABC). Median Ki-67 was 90% (range, 50,100%). 9/12 (75%) were positive for MYD88 L265P mutation. 13/21 (62%) were double expressor and 3/22 (14%) had double hit lymphoma. 37/45 (82%) had ECOG &lt; 2. LDH was elevated in 13/37 (35%). The median number of therapies was 1 (range; 1-11). The initial treatments were chemo-radiation (30%), chemo alone (38%), rituximab alone (11%), radiation (RT) alone (16%), rituximab-RT (2.5%), and surgical (2.5%). The median follow-up time for the whole cohort was 80 months (95% CI; 32, 169). The median progression free survival and overall survival (OS) were 20 months (95% CI; 12, 34) and 80 months (95% CI; 48, 119). 22/45 (49%) are alive. The cause of death was disease-related in 11/23 (48%). The median number of relapses was 1 (range; 0, 11) with (60%) relapsed after first line. 4/45 pts had autologous stem cell transplant after relapse. 7/45 (16%) had a systemic relapse at a median time of 35 months from diagnosis (range; 16, 112). Age above 60 years was associated with worse OS; 70 months (95% CI; 30, 99) vs NR (95% CI; 135, NR), p = 0.025. ECOG performance status &lt; 2 was associated with better OS, NR months (95% CI; 80, NR) vs 48 months (95% CI; 3.3, 88),p = 0.0003. 1 pt received Ibrutinib on 11th relapse and had 3 years of PFS. Conclusions: PCDLBCL is predominantly ABC type, affects elderly pts and is characterized by multiple cutaneous relapses followed in some cases by systemic relapses. Increased age and poor performance status had a negative impact on OS. High incidence of MYD88 L265P mutation is observed in PCDLBCL similar to immune-privileged site lymphomas.

Impact of Interim FDG-PET (iPET) in the Outcomes of Patients with Aggressive B-Cell Lymphomas Receiving DA-EPOCH-R

Introduction: 2-[18F] fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) remains the standard of care for baseline and end of treatment scans for aggressive non-Hodgkin lymphomas (NHLs). However, the role of interim FDG-PET remains not as well defined across aggressive NHLs, especially in the era of high-intensity chemoimmunotherapy. Interim FDG-PET (iPET) can serve as an early prognostic tool, and prior studies evaluating the utility of iPET-guided treatment strategies primarily focused on diffuse large B-cell lymphomas (DLBCL) and frontline R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). Classification criteria systems assessing response also differ between studies with no clear consensus between use of Deauville criteria (DC), International Harmonization Project (IHP), and the ΔSUVmax method. Methods: This study evaluates our institutional experience with iPET during treatment with DA-EPOCH ± R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin with or without Rituximab) in aggressive NHLs. We retrospectively evaluated 70 patients at Moffitt Cancer Center who started on DA-EPOCH ± R between 1/1/2014 to 12/31/2018 for aggressive NHLs. Response on interim and end-of-treatment (EOT) scans were graded per DC, IHP, and ΔSUVmax methods, and progression free survival (PFS) probability estimates were calculated with chi-square testing and Kaplan Meier method. PFS outcomes were compared between interim negative and positive scans based on each scoring method. Outcomes were also compared between groups based on interim versus EOT positive or negative scans. Results: We identified 70 patients with aggressive NHLs who received DA-EPOCH ± R at our institute. The most common diagnoses were DLBCL (61%) followed by Burkitt's lymphoma (10%), primary mediastinal B-cell lymphoma (9%), plasmablastic lymphoma (7%), gray zone lymphoma (6%), primary cutaneous large B-cell lymphoma (1%), primary effusion lymphoma (1%), and other high-grade NHL not otherwise specified (3%). Of the 43 patients with DLBCL, 21/43 (49%) had double hit lymphoma (DHL) while 7/43 (16%) had triple hit lymphoma (THL), and 3/43 (7%) had MYC-rearranged DLBCL while 2/43 (5%) had double expressor DLBCL. Thirty nine out of 70 (56%) were female, and median age at diagnosis was 58.39 years (range 22.99 - 86.86 years). Most patients had stage IV disease (49/70, 70%), and 43/70 (61%) had more than one extranodal site while 45/70 (64%) had IPI score ≥ 3. Forty-six out of 70 (66%) received central nervous system prophylaxis, most with intrathecal chemotherapy (44/70, 63%). Fifty-five out of 70 (79%) had iPET available while 6/70 (9%) had interim computerized tomography (CT) scans. Fifty-six out of 70 (80%) had EOT PET, and 4/70 (6%) had EOT CT scans. Sustained complete remission occurred in 46/70 (66%) after frontline DA-EPOCH ± R (CR1), and 12/70 (17%) were primary refractory while 5/70 (7%) had relapse after CR1. Four of 70 (6%) died before cycle 3, and 3/70 (4%) did not have long-term follow-up due to transition of care elsewhere. Median follow-up was 15.29 months (range 0.85 - 60.09 months). There was significantly better PFS observed if iPET showed DC 1-3 compared to DC 4-5 (Χ2=5.707, p=0.0169), and PFS was better if iPET was negative by IHP criteria (Χ2=4.254, p=0.0392) or ΔSUVmax method (Χ2=6.411, p=0.0113). Comparing iPET to EOT PET, there was significantly better PFS if iPET was negative with EOT PET negative (iPET-/EOT-) compared to iPET positive with EOT negative (iPET+/EOT-), and iPET+/EOT+ and iPET-/EOT+ had worse PFS after iPET-/EOT- and iPET+/EOT- respectively. This pattern in iPET/EOT PFS probability remained consistent when comparing DC (Χ2=30.041, p&lt;0.0001), IHP (Χ2=49.078, p&lt;0.0001), and ΔSUVmax method (Χ2=9.126, p=0.0104). These findings fit clinical expectations with positive EOT scans indicating primary refractory disease. There was no significant difference in PFS when comparing DLBCL versus non-DLBCL (Χ2=3.461, p=0.0628) or DHL/THL versus non-DHL/THL diagnoses (Χ2=2.850, p=0.0914). Conclusion: Our findings indicate a prognostic role of iPET during treatment with DA-EPOCH ± R for aggressive NHLs. Significant differences in PFS were seen when graded by DC, IHP, and ΔSUVmax methods used in prior studies and when comparing interim versus EOT response. Larger studies are needed to confirm these findings. Disclosures Bello: Celgene: Speakers Bureau. Shah:Novartis: Honoraria; AstraZeneca: Honoraria; Spectrum/Astrotech: Honoraria; Adaptive Biotechnologies: Honoraria; Pharmacyclics: Honoraria; Jazz Pharmaceuticals: Research Funding; Incyte: Research Funding; Kite/Gilead: Honoraria; Celgene/Juno: Honoraria. Sokol:EUSA: Consultancy. Chavez:Janssen Pharmaceuticals, Inc.: Speakers Bureau; Genentech: Speakers Bureau; Kite Pharmaceuticals, Inc.: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees.

Immunosuppression-associated primary cutaneous diffuse large B-cell lymphoma mimicking cellulitis in a woman with dermatomyositis

Immunosuppression-associated primary cutaneous diffuse large B-cell lymphoma mimicking cellulitis in a woman with dermatomyositis

Primary cutaneous diffuse large B-cell lymphoma, leg type

Primary cutaneous diffuse large B-cell lymphoma, leg type

Case Report of Cutaneous Large B-cell leg-type Lymphoma in Young Woman

Cutaneous diffuse B-cell lymphoma leg type is a rare entity, it represents approximately 5% of primary cutaneous lymphomas. It has a poor prognosis. We report case of t 58 years old patient with a cutaneous B cell lymphoma leg type with dual location at the upper and lower limbs and bone lysis. Histological examination of the skin biopsy showed diffuse lymphomatous proliferation dissecting collagen, made of large cells with abundant cytoplasm moderately ill-defined, ovoid nuclei and with a discreet nuclear irregularity. The immunohistochemical study revealed lymphoma cells phenotype B with a homogeneous expression of CD 20, a homogeneous expression of CD 3, CD 43 positive. Bcl 2 was positive, asthe Bcl 6 and Mum 1. CD 5, CD10, MPO, CD 34, and Tdt were negative. The Ki 67 was positivein90%. The staging showed extensive osteolysis of the lower end of the humerus and the ulna. The patient received 2 courses RCHOP, with faillure of treatment, and then she received one course of RDHAP before dying. The particularity of this observation consists in the age; our patient was younger than the average, and the double localization of lymphoma in both arm and leg, with osteolysis. We discuss also the difficulties of treatment of this disease.

Successful Treatment of Primary Cutaneous Diffuse Large B-Cell Lymphoma Leg Type With Single-Agent Venetoclax.

Successful treatment of primary cutaneous diffuse large B cell lymphoma leg type with single agent venetoclax

A Case of Primary Cutaneous Diffuse Large B-cell Lymphoma with Localized Cutaneous Nodular Amyloidosis

A Case of Primary Cutaneous Diffuse Large B-cell Lymphoma with Localized Cutaneous Nodular Amyloidosis

Primary cutaneous follicular center B-cell lymphoma with spontaneous regression in a Hispanic woman

Primary cutaneous follicular center B-cell lymphoma with spontaneous regression in a Hispanic woman

Advances in primary cutaneous marginal zone B-cell lymphoma

Primary cutaneous marginal zone B-cell lymphoma (pcMZL) belongs to mucosa-associated lymphoid tissue (MALT) lymphoma, and is a subtype of indolent non-Hodgkin lymphoma. Chronic antigen stimulation and gene mutations are key-players in its pathogenesis. Biopsy and immunohistochemistry are gold-standard examination for the diagnosis and staging of pcMZL. The overall prognosis of patients is excellent. This review will illustrate the advances in pathogenesis, diagnosis, therapy and prognosis of pcMZL, aimed to raise the recognition and making choice of treatments for this disease. Key words: Lymphoma, B-cell, marginal zone; Antigens; Mutation; Therapeutics

A Rare Case of Cutaneous Diffuse Large B-cell Lymphoma Presenting as a Chronic “Infectious” Skin Ulcer

Cutaneous diffuse large B-cell lymphoma (DLBCL) usually manifests as papules, nodules, or plaques. A rare case of a patient with a chronic skin ulcer and signs and symptoms of infection, including fever and large amounts of yellow wound exudate, is presented. Fifteen (15) months before diagnosis, a 43-year-old otherwise healthy man noted soreness without apparent cause in his upper chest and a palpable 2 cm x 2 cm focal lump. The patient developed frequent fevers, and the lump enlarged over time, producing purulent exudate. For 14 months, the patient was examined and treated at 5 hospitals, but biopsies, smears, cultures, and various types of nucleic acid testing were negative. Antibiotics to treat the suspected but nonclassified infection were ineffective. Ultimately, debridement and pathological examination of necrotic tissue from the deep sinus revealed DLBCL. The patient was provided chemotherapy, surgical debridement, and negative pressure wound therapy. Wounds started to reduce in size once chemotherapy was initiated. The wound was surgically closed with a split-skin graft, and the patient was discharged 93 days following admission to the authors' facility. This case illustrates the possibility of cutaneous DLBCL in patients with chronic skin ulcers and infectious manifestation that do not respond to antibiotic therapy. Prompt deep tissue debridement and pathological examination of deep tissue will help confirm the presence of cutaneous DLBCL and guide required chemotherapy.

Treatment of pediatric cutaneous lymphomas

Pediatric cutaneous lymphomas are a group of lymphoproliferative diseases with obvious heterogeneity, which are primarily derived from the skin. The classification is mainly based on the WHO-EORTC and WHO systems. The most common types of primary cutaneous lymphomas in children are cutaneous mature T-cell lymphomas (CTCL) and cutaneous mature B-cell lymphomas (CBCL) . Pediatric cutaneous lymphomas are rare, and their treatment approaches are mainly based on some case reports because of their clinical manifestations different from those of adults, few clinical trials and cautious medication. Treatments include topical drugs, phototherapy, radiotherapy, chemotherapy, surgery, stem cell transplantation and some new drugs. There are great differences in the therapeutic approaches and responses among various types of cutaneous lymphomas. Conservative treatment is effective in most childhood mycosis fungoides and other cutaneous lymphomas with inert biological features, while invasive cutaneous lymphomas still require chemotherapy and targeted therapies. Invasive cutaneous lymphomas occur more frequently in children than adults, so more attention should be paid to systematic assessment and follow-up observation. Key words: Lymphoma; Skin; Lymphoma, T-cell; Lymphoma, B-cell; Child; Therapeutic uses

Cutaneous EBV-associated B-cell lymphoma revealing an angioimmunoblastic T-cell lymphoma

Cutaneous EBV-associated B-cell lymphoma revealing an angioimmunoblastic T-cell lymphoma

A case of primary cutaneous diffuse large B-cell lymphoma-leg type complicated by bullous pemphigoid

A case of primary cutaneous diffuse large B-cell lymphoma-leg type complicated by bullous pemphigoid