Intravascular Lymphoma Research Articles

O-130 - Single-cell RNA sequencing reveals crosstalk between T cells and endothelial cells in intravascular T-cell lymphoma

O-130 - Single-cell RNA sequencing reveals crosstalk between T cells and endothelial cells in intravascular T-cell lymphoma

A Woman With Multifocal Ischemic Strokes and Progressive Cognitive Impairment due to Intravascular Lymphoma.

A Woman With Multifocal Ischemic Strokes and Progressive Cognitive Impairment due to Intravascular Lymphoma.

Intravascular Large B-Cell Lymphoma Detected by Cutaneous Biopsy with Pancytopenia and Negative Bone Marrow Biopsy

Intravascular large B-cell lymphoma is a B-cell lineage neoplasm, which is typically recognized as a high-grade neoplasm with poor outcomes. Though rare, the cutaneous variant can present with multiple lesions of the skin and negative systemic staging. We present a case of intravascular large B-cell lymphoma first identified on cutaneous biopsy, with pancytopenia, in addition to negative bone marrow biopsy and serum protein electrophoresis. The patient is a 71-year-old male who presented with upper body pruritis and pancytopenia, as well as weight loss and fatigue. A full body skin exam incidentally revealed a well-demarcated, firm, bound down atrophic lichenified plaque on the right anterior thigh. A punch biopsy revealed large, atypical lymphoid cells exclusively localized within the lumina of variably sized blood vessels, immunoreactive for CD20, BCL6, MUM1, and CD5, but negative for CD3, CD10, cyclin D1, and TdT. A diagnosis of intravascular large B-cell lymphoma was made, and he was referred to oncology for further treatment. Full body skin exam is a mainstay of dermatologic practice, and it is essential to pursue biopsy with uncharacteristic lesions, regardless of the presenting concern.

Progressive conus medullaris lesions are suggestive of intravascular large B-cell lymphoma.

Spinal cord lesions are observed in 40% of all central nervous system lesions in intravascular large B-cell lymphoma (IVLBCL). However, because IVLBCL is a very rare disease, its clinical features are not well defined, which may delay appropriate diagnosis and treatment, whilst the acute to subacute course of brain lesions in patients with IVLBCL is well established. Therefore, this study aimed to clarify the clinical features of spinal cord lesions in patients with IVLBCL. The medical records of patients with IVLBCL admitted to our hospital between 2010 and 2020 were searched. The inclusion criteria were preceding neurological symptoms without non-neurological symptoms and pathologically confirmed IVLBCL in various organs. Clinical features of spinal cord involvement in patients with IVLBCL were assessed and distinguished from those of brain involvement. Sixteen consecutive patients with IVLBCL were divided into two groups: six patients with spinal involvement (spinal cord type) and 10 patients with brain involvement (brain type). In the spinal cord type, four patients had chronic progression and two had subacute progression. Acute progression (0% vs. 80.0%) and sudden onset (0% vs. 50.0%) occurred significantly less frequently in the spinal cord than in the brain. All spinal cord lesions involved the conus medullaris. Spinal cord involvement in IVLBCL has a predominantly chronic progressive course that is exclusive to brain involvement. Conus medullaris lesions are suggestive of IVLBCL and are useful for early and accurate diagnosis and treatment.

FIVE‐YEAR RESULTS OF A PHASE 2 STUDY OF CNS‐ORIENTED THERAPY WITH R‐CHOP FOR UNTREATED INTRAVASCULAR LARGE B‐CELL LYMPHOMA: FINAL ANALYSIS OF THE PRIMEUR‐IVL STUDY

Introduction: Intravascular large B-cell lymphoma (IVLBCL) is a rare disease entity of extranodal large B-cell lymphoma characterized by selective growth of lymphoma cells in the lumina of small vessels. The prognosis of IVLBCL is typically poor without timely diagnosis. Based on the promising efficacy of rituximab-containing chemotherapy and a high incidence of secondary central nervous system (CNS) involvement after rituximab-chemotherapy, we conducted the PRIMEUR-IVL study to explore the efficacy of CNS-oriented therapy with rituximab-chemotherapy whose primary analysis demonstrated 2-year progression-free survival (PFS) of 76% and 2-year overall survival (OS) of 92% with a low incidence of secondary CNS involvement of 3%. Patients and Methods: The PRIMEUR-IVL study is a multicenter, single-arm, phase 2 trial at 22 hospitals in Japan for untreated histologically confirmed IVLBCL patients without apparent CNS involvement at diagnosis. The treatment regimen includes three cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) followed by two cycles of rituximab with high-dose methotrexate and additional three cycles of R-CHOP. Intrathecal chemotherapy including methotrexate, cytarabine, and prednisolone was administered four times during the R-CHOP phase. We present a preplanned final analysis of the PRIMEUR-IVL study including 5-year PFS, OS and secondary CNS involvement. Results: A total of 38 patients were enrolled, of whom 37 patients were eligible. One patient with a history of testicular lymphoma was excluded. As of July 2021, with a median follow-up of 7.1 years (interquartile range 5.6–8.7), 5-year PFS in all eligible patients was 68% (95% confidence interval [CI] 50%–80%) and OS was 78% (95% CI 61%–89%). Median PFS and OS were not reached. No additional secondary CNS involvement was observed after primary analysis with 3% of cumulative incidence. Severe adverse events after the primary analysis were grade 4 neutropenia (n = 1) and grade 4 myelodysplastic syndrome that did not require specific treatment (n = 1). During the observation period after enrollment, there were a total of eight deaths due to following reasons: primary disease (n = 6), sepsis (n = 1), and unknown sudden death (n = 1). Conclusions: In this 5-year, long-term follow-up analysis of the PRIMEUR-IVL study, frontline treatment of CNS-oriented therapy with standard R-CHOP for untreated IVLBCL patients without apparent CNS involvement provided durable response and clinically meaningful results for PFS, OS, and low incidence of secondary CNS involvement with manageable toxicity. Our result provides one of active treatment for untreated IVLBCL patients. The research was funded by: The Practical Research for Innovative Cancer Control, the Japan Agency for Medical Research and Development (AMED), Japan, JP19ck0106511 and the National Cancer Center Research and Development Fund, 23-A-17 and 26-A-4. The research was supported by the Center for Supporting Hematology-Oncology Trials (C-SHOT). Keyword: chemotherapy Conflicts of interests pertinent to the abstract K. Shimada Honoraria: AstraZeneca, Chugai, Eisai, Kyowa Kirin, Symbio M. Yamaguchi Honoraria: AbbVie, Bristol Myers Squibb, Chugai, Janssen, Kyowa Kirin, Meiji Seika, MSD, Nippon Shinyaku, SymBio, Takeda Research funding: AstraZeneca, Chugai Pharma, Genmab, Incyte, Eisai, Takeda, Nippon Shinyaku, Otsuka, Asahi Kasei, Kyowa Kirin, Chugai H. Nagai Honoraria: Kyowa Kirin, Takeda, Chugai, Bristol Myers Squibb, Eisai, Novartis, Janssen, Mundi Pharma, Lilly, AstraZeneca, Ono, Meiji, AbbVie, Nippon Shinyaku, GSK, Sumitomo Pharma, Genmab, CSL Behring Research funding: Kyowa Kirin, Takeda, Chugai, Bristol Myers Squibb, Janssen, AstraZeneca, Ono, AbbVie, Nippon Shinyaku, Daiichi-Sankyo, Zenyaku Kogyo, Solasia J. Takizawa Honoraria: Chugai, Kyowa Kirin N. Fukuhara Honoraria: Chugai, Genmab, AbbVie, Takeda, Eli Lilly, AstraZeneca, Meiji Seika, Ono, Janssen, Bristol-Myers Squibb, Eisai, Kyowa Kirin, Symbio, Novartis Research funding: Chugai, Genmab, AbbVie, Takeda, Eli Lilly, Incyte, Chordia Therapeu D. Ennishi Honoraria: Eisai, Kyowa Kirin, Chugai Research funding: Nippon Shinyaku, Chugai Y. Minami Consultant or advisory role: Takeda, Novartis Honoraria: Bristol-Myers Squibb, Novartis, Pfizer, Daiichi Sankyo, Astellas Research funding: CMIC, Bristol-Myers Squibb, Novartis, Takeda, Chugai, Ono Y. Atsuta Consultant or advisory role: Meiji Seika, JCR Pharmaceuticals, Kyowa Kirin Honoraria: Novartis, AbbVie H. Kiyoi Honoraria: Novartis, Astellas, AbbVie, Chugai Research funding: Zenyaku Kogyo, Nippon Shinyaku, Chugai, Astellas, Kyowa Kirin, Takeda, Sumitomo Pharma, Sanofi, Eisai, Ono, FUJIFILM, Kyowa Kirin, Otsuka, Perseus Proteomics, Daiichi Sankyo, CURED, Bristol-Myers Squibb, AbbVie

What is new about primary cutaneous B cell lymphomas.

Primary cutaneous B-cell lymphomas (CBCL) are a heterogeneous group of B-cell lymphomas without evidence of extracutaneous disease at the time of diagnosis. The 2022 World Health Organization classification of mature lymphoid neoplasms differentiates the indolent primary cutaneous marginal zone lymphoproliferative disorder, primary cutaneous follicle center lymphoma and Epstein-Barr virus-positive mucocutaneous ulcer, from the more aggressive primary cutaneous diffuse large B-cell lymphoma, leg-type and intravascular large B-cell lymphoma. The new updates in the 2022 classification are based on recent scientific advances in the understanding and characterization of these entities. This article aims to review the main clinical, cellular and molecular features of the five CBCL subsets along with their management and treatment. The exponentially growing evidence for new treatment options for systemic B-cell lymphomas raises expectations for the field of CBCL as well. However, specific prospective high quality research on CBCL is still crucial to further define their management and update international guidelines.

Anti-transcriptional intermediary factor 1γ antibody-positive dermatomyositis with intravascular lymphoma.

Anti-transcriptional intermediary factor 1γ antibody-positive dermatomyositis with intravascular lymphoma.

Clinicopathological characteristics associated with the engraftment of patient lymphoma cells in NOG mice.

Patient-derived xenograft (PDX) mouse models are useful for deepening our understanding of the biology of malignant lymphoma; however, factors associated with the success of the PDX lymphoma model are largely unknown. We retrospectively analyzed the characteristics of 66 xenotransplantations from 65 patients. In all, 43 (65%) specimens were obtained from patients aged > 60years, and 42 (64%) specimens were obtained at diagnosis. Specimens were obtained from patients with the following diseases: diffuse large B-cell lymphoma (n = 30), intravascular large B-cell lymphoma (n = 12), follicular lymphoma (n = 8), peripheral T-cell lymphoma (n = 7), mantle cell lymphoma (n = 2), and other (n = 7). The specimens were sourced mainly from bone marrow (n = 31, 47%) and extranodal tumors (n = 13, 20%). Engraftment was successful in 33/66 (50%) xenotransplantations. The median age of patients who provided successful specimens was significantly higher than that for unsuccessful specimens (p = 0.013). Specimens with a high proportion of tumor cells in the graft and those obtained from patients with relapsed/refractory disease showed higher tendencies toward successful engraftment. Taken together, these data suggest that tumor cells with a highly malignant potential might have a high likelihood of engraftment.

Intravascular Large B-cell Lymphoma Presenting as Rapidly Progressive Encephalopathy

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin’s lymphoma that selectively involves the lumina of vessels, and may present as ischemic stroke. A 62-year-old man presented with progressive aphasia, dementia, seizure and stroke-like symptom. Brain magnetic resonance imaging revealed dynamic progressive fluid attenuated inversion recovery lesions and multiple punctate microbleeds in the bilateral subcortical region. A kidney biopsy revealed IVLBCL. We report a patient with IVLBCL presenting as rapidly progressive encephalopathy.

Intravascular CNS Lymphoma: An elusive diagnosis (P2-5.016)

<h3>Objective:</h3> To report a rare case of intravascular CNS lymphoma presenting with subacute neuropsychiatric symptoms and multifocal cerebral infarcts and hemorrhages. <h3>Background:</h3> Intravascular lymphoma is a rare and aggressive subtype of extra-nodal non-Hodgkin lymphoma. It is characterized by proliferation of neoplastic B-lymphocytes within the lumen of small blood vessels, especially capillaries and post-capillary venules. Presentation is variable and depends on the organ dysfunction due to small vessel occlusion. Most common organs involved are CNS, skin and lungs. Given the rarity of the disease, variable presentation and absence of an extravascular tumor mass or peripheral blood abnormalities, the diagnosis can be extremely challenging and is often delayed. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 62-year old healthy woman presented with transient left hemiplegia and left facial numbness and was diagnosed with transient ischemic attack. In one month, she developed dysarthria and left hemibody clonic seizures without any systemic complaints. Over the next four months, she had a step-wise decline and developed severe apathy, decreased verbal fluency, diffuse hypertonia and worsening encephalopathy. Serial brain MRIs over four months showed acute-subacute infarcts and intracerebral hemorrhages involving both anterior and posterior circulation bilaterally with scattered micro-hemorrhages. Extensive work up, including CT-angiogram and PET-CT were unrevealing. CSF was notable for high protein level without pleocytosis and negative infectious and autoimmune workup. DSA was unrevealing. She did not respond to high dose steroids, intravenous immunoglobulin and cyclophosphamide. Brain biopsy revealed CNS intravascular large B-cell lymphoma. Patient was started on chemotherapy with HyperCVAD-R protocol with no improvement. <h3>Conclusions:</h3> Intravascular CNS lymphoma is a challenging diagnosis. It presents as subacute-chronic CNS vasculopathy with multifocal infarcts and hemorrhages and can mimic other CNS vasculopathies, including primary CNS angiitis and amyloid-related angiopathy. Tissue biopsy is the diagnostic gold standard and should be considered early if poor response to immunotherapy is noted. Despite high-dose chemotherapy, prognosis is poor. <b>Disclosure:</b> Dr. Ticku has nothing to disclose. Dr. Serra has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen Idec. Dr. Serra has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Bristol Myers Squibb.

A case of intravascular large B-cell lymphoma – Our clinical experience

Fever of unknown origin is always a diagnostic challenge in establishing etiology. A gentleman in his 70s presented with complaints of fever and dry cough for 2 months duration. We proceeded with contrast imaging of the thorax and abdomen which revealed mild hepatomegaly. Bone marrow examination with bone marrow culture and 18-fluorodeoxyglucose positron emission tomography/computed tomography (18-FDG PET/CT). Bone marrow aspirate smears were hypercellular with around 50% atypical lymphoid cells with plasmacytoid and bizarre morphology with few multilobed nuclei. Bone marrow biopsy revealed predominantly sinusoidal involvement by neoplastic cells. On immunohistochemistry, tumor cells were positive for CD45, CD79a, CD20, and MUM1 and were negative for CD5, CD10, and BCL6. Ki-67 was around 60% in tumor cells. FDG PET/ CT revealed diffusely increased uptake in the both axial and appendicular skeleton with (SUVmax 5.06) and diffusely increased FDG uptake (SUVmax 3.67) noted in the spleen. As intravascular large B-cell lymphoma is a highly aggressive non-Hodgkin lymphoma with a high risk of central nervous system involvement, we treated it with chemoimmunotherapy (R-CHOP) with intrathecal methotrexate. After a clinical follow of 3 months, the patient developed relapsed with a soft-tissue swelling over the right leg. The patient was treated with two cycles of R-DHAP and had progressive disease and started on Ibrutinib, Lenalidomide, and Rituximab (2 cycles). Post two cycles, the patient had progressive disease and switched to acalabrutinib based therapy. After 1 month of acalabrutinib-lenalidomide-rituximab therapy, the patient had disease progression and succumbed to the disease.

Intravascular Large B-cell Lymphoma Presenting as Sudden Death.

Intravascular large B-cell lymphoma is a rare subset of non-Hodgkin lymphoma composed of mature B lymphoma cells confined to the intravascular space. This disease remains elusive because it lacks a discrete tumor mass, can affect any part of the body, and has vague symptoms paired with heterogeneous clinical findings resulting in delayed or missed accurate diagnosis, even at postmortem examination. This is a case of a woman who died within hours of presenting to the emergency department with a diagnosis of intravascular large B-cell lymphoma made through autopsy examination, adding to the knowledge of this rare disease and bringing it to the attention of practicing autopsy and forensic pathologists.

Intravascular large B-cell lymphoma appearance on dual-energy computed tomography: a case report

BackgroundIntravascular large B-cell lymphoma (IVLBCL) is the proliferation of neoplastic B lymphocytes in the vascular space. Since conventional computed tomography (CT) shows nonspecific findings, differentiation between IVLBCL and other lung diseases, such as diffuse interstitial lung disease, is difficult.Case presentationA 73-year-old man presented with dyspnea and hypoxemia. Laboratory findings showed an increased lactate dehydrogenase level of 1690 U/L (normal: 130–235 U/L) and soluble interleukin-2 receptor level of 1140 U/mL (normal: 157–474U/mL). Dual-energy CT iodine mapping showed a significant symmetrical decrease in iodine distribution in the upper lungs, suggesting an unusual distribution of pulmonary hypoperfusion. Therefore, IVLBCL was suspected. A random skin biopsy confirmed the diagnosis of IVLBCL. Due to the severity of the disease, lung biopsy was averted. After admission to the hospital, high-dose methotrexate was administered for central nervous system involvement, due to findings of suspected intracranial infiltration on a brain magnetic resonance imaging and elevated cell counts on lumbar puncture. Subsequently, oxygen demand improved, and rituximab along with cyclophosphamide, doxorubicin, vincristine, and prednisone was added to the patient’s regime. Eventually, oxygen administration was terminated, the patient’s general condition improved, and the patient was discharged after 47 days of hospitalization.ConclusionsSince the diagnosis of IVLBCL depends on whether it is possible to suspect IVLBCL, the finding of decreased iodine perfusion demonstrated on dual-energy CT is considered important information for diagnosis. An immediate diagnosis of IVLBCL is needed to avoid rapid disease progression and introduce early treatment for a favorable prognosis. In this case, unique pulmonary hypoperfusion demonstrated by dual-energy CT promoted early diagnosis of IVLBCL.

415 Predictive factors for the diagnosis of intravascular large B-cell lymphoma among suspicious cases: A single-center retrospective analysis

415 Predictive factors for the diagnosis of intravascular large B-cell lymphoma among suspicious cases: A single-center retrospective analysis

Intravascular Large B-Cell Lymphoma Identified by PET/CT.

Intravascular large B-cell lymphoma is a rare subtype of extranodal invasive non-Hodgkin lymphoma, and the diagnosis is challenging. Here we report findings of 18F-FDG PET/CT study in a case of intravascular large B-cell lymphoma invading bilateral lungs and kidneys in a 63-year-old woman. The PET/CT images showed diffuse increased FDG uptake in bilateral lungs and kidneys. Unexpectedly, there was no abnormal density on the CT images. The 18F-FDG PET/CT seems to be valuable and sensitive in the diagnosis of intravascular large B-cell lymphoma.

Abstract 5521: Racial and ethnic disparities for intravascular large B-cell lymphoma: A Surveillance, Epidemiology, and End Results (SEER) database analysis with emphasis on Hispanics

Abstract Background: Intravascular large b-cell lymphoma (IVLBCL) is a rare but highly aggressive subtype of non-Hodgkin lymphoma (NHL). IVLBCL is a clonal proliferation with selective growth within the blood vessel. It has been primarily described in Asian and European populations, and a median age presentation is in the sixth to seventh decades without a sex predilection. The US incidence is 0.95 in 1 million, but is thought to be higher, as the diagnosis is mainly done postmortem. Meanwhile, the incidence rate of IVBCL in Hispanics (HI) remained unknown. This study looks at demographics, treatment patterns, and outcomes of patients with IVLBCL in the US, examining disparities by HI vs Non-Hispanic (NH). Methods: Data were analyzed on IVLBCL patients reported to the SEER 18 database between 2000 and 2018. SEER 18 contains the most comprehensive population-based cancer information in the US, covering approximately 27% of the US population and 36% of HI alone. The racial groups analyzed were NH whites, HI whites, blacks, and Asians/PIs (Pacific Islanders). Patient characteristics, age-adjusted incidence rate, and survival rate were compared across ethnic groups. Kaplan-Meier and Cox regression analyses compared overall survival (OS) between HI and NH. Multivariate analysis and propensity score matching were performed, with adjustment for age, stage and B-symptoms. Results: We identified 164 patients with IVLBCL, of which 10% were HI. 41% of HI were male vs 44% of NH. HI were diagnosed at an older age, 72. vs 69 y.o., compared to NHI (p=0.907). Most of NH and HI were diagnosed between 60-80 y.o. (p=0.322), 53.1% and 47.1%, respectively. Regarding race, HI and NH were mainly identified as whites (88% vs 78%), followed by Asians (6% vs 17%) (p=0.013). For HI, 53% presented B symptoms compared to 16% of NH (p=&amp;lt;0.001). On survival analysis, the survival probability at 2, 5 and 10 years of HI vs NH was (0.540 vs 0.505), (0.432 vs 0.425), and (0.443 vs 0.237), respectively. The median survival time was 0.8 years for HI and 1.9 years for NH. The 10 year OS probability was not significantly different for HI vs NH (p=0.66).On multivariate analysis, when adjusted for age, those patients who were 60 to 80 y.o. had worse OS compared to those younger than 60 y.o., with HR 1.4 (95% CI: 0.7 - 2.1). Conclusion: In our study, despite a lower percentage of patients identified as Asian in our HI population, there is a significant difference in the presence of B-symptoms between HI and NH, going against the traditional description of an Asian variant with increased systemic symptoms. Despite this finding, it demonstrated similar outcomes in the 10 years survival analysis for HI and NH. Standardized treatment may explain why no variation was reported in OS. Our analysis shows that ethnic variations do not seem to affect oncological outcomes in IVLBCL for HI in the US. Citation Format: Daniela Urueta Portillo, Daniel Rosas, Joel E. Michalek, Qianqian Liu, Adolfo E. Diaz Duque. Racial and ethnic disparities for intravascular large B-cell lymphoma: A Surveillance, Epidemiology, and End Results (SEER) database analysis with emphasis on Hispanics. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 5521.

Ocular Involvement in a Patient with Intravascular Large B-Cell Lymphoma: A Diagnostic Challenge. Clinical Case Report and Literature Review

ABSTRACT Purpose To describe the ophthalmic findings and diagnosis of a case of intravascular large B-cell lymphoma. Methods Clinical case observational report. Clinical case A Spanish 51-year-old man referred to our hospital with a diagnosis of panuveitis. The patient presented with blurred vision, photophobia, fever, and weight loss. Ocular examination revealed anterior uveitis, vitritis, and multiple round and oval creamy spots on the posterior pole. Fluorescein angiography, optical coherence tomography (OCT), and angio-OCT were used for the ocular examination. The diagnosis of lymphoma was formulated on the basis of a random normal skin biopsy, which showed significant CD20 cellularity within the vessels and extensive CD3 expression. Conclusions Intravascular lymphoma is a rare form of extranodal diffuse large B-cell lymphoma, often with delayed diagnosis because of the nonspecific symptoms. Hence, random skin biopsy could be useful in the diagnosis.

Intravascular large B-cell lymphoma diagnosed at autopsy

Background: Intravascular large B-cell lymphoma is a rare, extranodal lymphoma characterised by intravascular growth of a malignant mature B-cell population, primarily affecting capillaries. Pre-mortem diagnosis can be challenging as patients often present with non-specific clinical findings including fever of unknown origin, decreased general condition and neurological abnormalities. Radiological findings may be non-specific or falsely negative. Aims: We present the case of an 87-year-old lady who presented to hospital with cognitive and functional decline, headache and presumed transient ischaemic attacks. Despite extensive investigations a definitive cause for her symptoms was unable to be established prior to her death. Autopsy revealed multiorgan involvement by intravascular large B-cell lymphoma. Methods: The patient underwent a full non-coronial hospital autopsy. With consent, the patient’s brain was retained for extended fixation and neuropathological assessment which also revealed widespread lymphomatous infiltration. Results and conclusions: This case demonstrates a rare case of intravascular large B-cell lymphoma diagnosed at autopsy and highlights the difficulties in pre-mortem diagnosis due to the non-specific clinical presentation and lack of diagnostic radiological features.

The great masquerader, Whipple’s disease a rare bacterial infection – a case report

Background: Whipple’s disease is a rare bacterial infection caused by bacterium Trophyrema Whipplei that most often affects digestive system mainly duodenum and joints. Whipple’s disease interferes with normal digestion by impairing the breakdown of foods and hampering body’s ability to absorb nutrients such as fats and carbohydrates. Histopathological characteristics overlap with other diseases (such as mycobacterial infections, enteropathy associated T-cell lymphoma, inflammatory bowel disease) which poses diagnostic challenges. We report a case 59-year-old male who presented with pyrexia of unknown origin (PUO) of 10 months duration. At the time of diagnosis, T cell lymphoma, intravascular lymphoma, enteropathy associated T cell lymphoma (EATL) and inflammatory bowel disease were under active consideration. A duodenal biopsy was sent for histopathological examination which showed enlarged villi filled by foamy macrophages which raised suspicion of WD and was further confirmed by PCR. Conclusion: This case is noteworthy because it is highly unusual and extremely rare presentation as PUO. This led to a delay in diagnosis of some 10 months, with numerous differential diagnoses investigated in the interim. Of the more common symptoms, the patient did have arthralgia (attributed to rheumatoid arthritis), but no weight loss, diarrhea or abdominal pain.

Intravascular Large B-Cell Lymphoma: Two Cases Observed at a Single Institution

Intravascular large B-cell Lymphoma (IVLBCL) is a rare subtype of extranodal non-Hodgkin’s lymphoma that is challenging to diagnose and has a poor prognosis. Here, we describe two patients newly diagnosed with IVLBCL at our institution: an African man with hemophagocytic-syndrome-associated IVLBCL and an Italian woman with a cutaneous variant of IVLBCL. They presented with very different clinical manifestations. Both cases were diagnosed in a timely manner, successfully treated, and achieved long-lasting remissions.