Publications by authors named "Giovanni Martino"

88 Publications

Primary effusion lymphoma occurring in the setting of transplanted patients: a systematic review of a rare, life-threatening post-transplantation occurrence.

BMC Cancer 2021 Apr 27;21(1):468. Epub 2021 Apr 27.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100, Terni, Italy.

Background: Primary effusion lymphoma is a rare, aggressive large B-cell lymphoma strictly linked to infection by Human Herpes virus 8/Kaposi sarcoma-associated herpes virus. In its classic form, it is characterized by body cavities neoplastic effusions without detectable tumor masses. It often occurs in immunocompromised patients, such as HIV-positive individuals. Primary effusion lymphoma may affect HIV-negative elderly patients from Human Herpes virus 8 endemic regions. So far, rare cases have been reported in transplanted patients. The purpose of our systematic review is to improve our understanding of this type of aggressive lymphoma in the setting of transplantation, focusing on epidemiology, clinical presentation, pathological features, differential diagnosis, treatment and outcome. The role of assessing the viral serological status in donors and recipients is also discussed.

Methods: We performed a systematic review adhering to the PRISMA guidelines. The literature search was conducted on PubMed/MEDLINE, Web of Science, Scopus, EMBASE and Cochrane Library, using the search terms "primary effusion lymphoma" and "post-transplant".

Results: Our search identified 13 cases of post-transplant primary effusion lymphoma, predominantly in solid organ transplant recipients (6 kidney, 3 heart, 2 liver and 1 intestine), with only one case after allogenic bone marrow transplantation. Long-term immunosuppression is important in post-transplant primary effusion lymphoma commonly developing several years after transplantation. Kaposi Sarcoma occurred in association with lymphoma in 4 cases of solid organ recipients. The lymphoma showed the classical presentation with body cavity effusions in absence of tumor masses in 10 cases; 2 cases presented as solid masses, lacking effusions and one case as effusions associated with multiple organ involvement. Primary effusion lymphoma occurring in the setting of transplantation was more often Epstein Barr-virus negative. The prognosis was poor. In addition to chemotherapy, reduction of immunosuppressive treatment, was generally attempted.

Conclusions: Primary effusion lymphoma is a rare, but often fatal post-transplant complication. Its rarity and the difficulty in achieving the diagnosis may lead to miss this complication. Clinicians should suspect primary effusion lymphoma in transplanted patients, presenting generally with unexplained body cavity effusions, although rare cases with solid masses are described.
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http://dx.doi.org/10.1186/s12885-021-08215-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8077837PMC
April 2021

B19 Infection in a Patient with Common Variable Immunodeficiency.

Mediterr J Hematol Infect Dis 2021 1;13(1):e2021026. Epub 2021 Mar 1.

Pathology Unit, Azienda Ospedaliera S. Maria di Terni, University of Perugia, 05100 Terni, Italy.

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http://dx.doi.org/10.4084/MJHID.2021.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7938927PMC
March 2021

Mycosis Fungoides with Dermal Mucin Deposition: An Unusual Variant of the Most Challenging Primary Cutaneous Lymphoma.

Case Rep Dermatol 2021 Jan-Apr;13(1):7-11. Epub 2021 Jan 14.

Pathology Unit, Ospedale di Terni, University of Perugia, Terni, Italy.

Mycosis fungoides (MF) is defined as an epidermotropic primary cutaneous T-cell lymphoma composed of small-to-medium-sized T lymphocytes with cerebriform nuclei and with a T-helper phenotype. LeBoit first described an unusual variant of MF with dermal acid mucin deposition. Such a variant was still considered in the list of clinicopathological variants of MF by Cerroni and colleagues. We herein report a case of patch-stage MF with abundant papillary dermal mucin deposition in a clinical setting of an erythematous patch on the lower abdomen and thigh.
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http://dx.doi.org/10.1159/000509525DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7879304PMC
January 2021

Primary Pulmonary B-Cell Lymphoma: A Review and Update.

Cancers (Basel) 2021 Jan 22;13(3). Epub 2021 Jan 22.

Pathology Unit, Azienda Ospedaliera S. Maria di Terni, University of Perugia, 05100 Terni, Italy.

Primary pulmonary B-cell lymphomas (PP-BCLs) comprise a group of extranodal non-Hodgkin lymphomas of B-cell origin, which primarily affect the lung without evidence of extrapulmonary disease at the time of diagnosis and up to 3 months afterwards. Primary lymphoid proliferations of the lung are most often of B-cell lineage, and include three major entities with different clinical, morphological, and molecular features: primary pulmonary marginal zone lymphoma of mucosa-associated lymphoid tissue (PP-MZL, or MALT lymphoma), primary pulmonary diffuse large B cell lymphoma (PP-DLBCL), and lymphomatoid granulomatosis (LYG). Less common entities include primary effusion B-cell lymphoma (PEL) and intravascular large B cell lymphoma (IVLBCL). A proper workup requires a multidisciplinary approach, including radiologists, pneumologists, thoracic surgeons, pathologists, hemato-oncologists, and radiation oncologists, in order to achieve a correct diagnosis and risk assessment. Aim of this review is to analyze and outline the clinical and pathological features of the most frequent PP-BCLs, and to critically analyze the major issues in their diagnosis and management.
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http://dx.doi.org/10.3390/cancers13030415DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7865219PMC
January 2021

Systemic Mastocytosis Associated with "Smoldering" Multiple Myeloma.

Diagnostics (Basel) 2021 Jan 7;11(1). Epub 2021 Jan 7.

Hematology Unit, CREO, Azienda Ospedaliera di Perugia, University of Perugia, 06129 Perugia, Italy.

A 79-year-old woman presented with a long history of peripheral eosinophilia. Previous right hemicolectomy for colonic polyposis was reported. Laboratory tests were notable for mild macrocitic anaemia and eosinophilia. β2 microglobulin and serum tryptase levels were elevated. Serum immunofixation revealed IgA/kappa monoclonal protein. Bence-Jones protein was positive. Bone marrow (BM) biopsy revealed the coexistence of two neoplastic components. Cohesive clusters of bland-looking, spindle-shaped mast cells, representing 20% of marrow cellularity, were close to aggregates of mature plasma cells occupying 40% of marrow cellularity. Molecular analysis on marrow aspirate demonstrated KIT D816V mutation, TET2 mutation, monoallelic deletion of TP53/17p13 and trisomy of ATM/11q23. A bone density study revealed mild osteoporosis. Full skeletal X-rays and magnetic resonance imaging (MRI) of spine and hips showed multiple, small rarefaction areas and an old L1-L2 fracture, both ascribed to osteoporosis. The association of systemic mastocytosis (SM) and multiple myeloma (MM) is very uncommon. The coexistence of SM with MM placed our patient in the SM with associated clonal haematological non-mast-cell lineage disease (SM-AHN) subtype. Midostaurin therapy was started.
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http://dx.doi.org/10.3390/diagnostics11010088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7825792PMC
January 2021

Neuromechanical Assessment of Activated vs. Resting Leg Rigidity Using the Pendulum Test Is Associated With a Fall History in People With Parkinson's Disease.

Front Hum Neurosci 2020 9;14:602595. Epub 2020 Dec 9.

W.H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Emory University, Atlanta, GA, United States.

Leg rigidity is associated with frequent falls in people with Parkinson's disease (PD), suggesting a potential role in functional balance and gait impairments. Changes in the neural state due to secondary tasks, e.g., activation maneuvers, can exacerbate (or "activate") rigidity, possibly increasing the risk of falls. However, the subjective interpretation and coarse classification of the standard clinical rigidity scale has prohibited the systematic, objective assessment of resting and activated leg rigidity. The pendulum test is an objective diagnostic method that we hypothesized would be sensitive enough to characterize resting and activated leg rigidity. We recorded kinematic data and electromyographic signals from rectus femoris and biceps femoris during the pendulum test in 15 individuals with PD, spanning a range of leg rigidity severity. From the recorded data of leg swing kinematics, we measured biomechanical outcomes including first swing excursion, first extension peak, number and duration of the oscillations, resting angle, relaxation index, maximum and minimum angular velocity. We examined associations between biomechanical outcomes and clinical leg rigidity score. We evaluated the effect of increasing rigidity through activation maneuvers on biomechanical outcomes. Finally, we assessed whether either biomechanical outcomes or changes in outcomes with activation were associated with a fall history. Our results suggest that the biomechanical assessment of the pendulum test can objectively quantify parkinsonian leg rigidity. We found that the presence of high rigidity during clinical exam significantly impacted biomechanical outcomes, i.e., first extension peak, number of oscillations, relaxation index, and maximum angular velocity. No differences in the effect of activation maneuvers between groups with clinically assessed low rigidity were observed, suggesting that activated rigidity may be independent of resting rigidity and should be scored as independent variables. Moreover, we found that fall history was more common among people whose rigidity was increased with a secondary task, as measured by biomechanical outcomes. We conclude that different mechanisms contributing to resting and activated rigidity may play an important yet unexplored functional role in balance impairments. The pendulum test may contribute to a better understanding of fundamental mechanisms underlying motor symptoms in PD, evaluating the efficacy of treatments, and predicting the risk of falls.
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http://dx.doi.org/10.3389/fnhum.2020.602595DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7756105PMC
December 2020

Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract: a tricky diagnosis of a gastric case.

BMC Gastroenterol 2020 Oct 14;20(1):336. Epub 2020 Oct 14.

Pathology Unit, Ospedale Di Terni, University of Perugia, Perugia, Italy.

Background: Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract is a rare low-grade clonal lymphoid proliferation, included as a provisional entity in the current World Health Organization classification. The disease is generally localized to the gastrointestinal tract, mainly small bowel and colon. Involvement of other organs is infrequently reported. The majority of patients show a protracted clinical course with persistent disease. A prolonged survival, even without treatment, is common.

Case Presentation: A 28-year-old woman had a 2-year history of dyspepsia and lactose intolerance. Autoimmune diseases and celiac disease were excluded. No gross lesions were identified by endoscopy. Multiple gastric biopsies showed a small-sized lymphoid infiltrate, expanding the lamina propria, with a non-destructive appearance. The lymphoid cells were positive for CD3, CD4, CD5, CD7 and negative for CD20, CD8, CD56, CD103, PD1, CD30, ALK1, CD10, BCL6, perforin, TIA-1, Granzyme B and Epstein-Barr virus-encoded RNA. KI-67 index was low (5%). Molecular analysis revealed a clonal T-cell receptor γ rearrangement. Bone marrow was microscopically free of disease, but molecular testing identified the same T-cell receptor γ rearrangement present in the gastric biopsies. After the diagnosis of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract, the patient received steroid therapy, only for 2 months. She is alive, with a stable disease restricted to the stomach, at 12 months from diagnosis.

Conclusions: Indolent T-cell lymphoproliferative disorder is usually a disease of adulthood (median age: 51 yrs). The small bowel and colon are the sites most commonly involved. Our case occurred in a young woman and affected the stomach, sparing small intestine and colon. Clonality testing identified involvement of bone marrow, a site infrequently affected in this disease. Our aim is focusing on the main diagnostic issues. If appropriate immunostainings and molecular analysis are not performed, the subtle infiltrate may be easily overlooked. The risk of misdiagnosis as more aggressive lymphomas, causing patient overtreatment, needs also to be considered.
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http://dx.doi.org/10.1186/s12876-020-01488-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7557083PMC
October 2020

Oral plasmablastic lymphoma: A case report.

Medicine (Baltimore) 2020 Sep;99(39):e22335

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

Introduction: Plasmablastic lymphoma (PBL) is an uncommon and aggressive large B-cell lymphoma commonly diagnosed in human immunodeficiency viruses -positive patients. Oral cavity is the most commonly PBL affected site. Most oral PBLs presented as asymptomatic swellings, frequently associated with ulcerations and bleeding. Most cases lacked B-symptoms, suggesting a more local involvement of the disease. No standard treatment is yet for oral PBL. Five-year survival rate recorded no more than 33.5%.

Patient Concerns: A 39-year-old male presented to Dental Clinic with 1 month swelling of the oral cavity, in absence of any other symptoms or signs. He followed antibiotic therapy just on suspicion of an oral abscess and later oral surgical treatment on suspicion of bone neoplasm.

Diagnosis: Surgical specimen analysis highlighted a diffuse infiltrate of large-sized atypical cells with plasmablastic appearance and plasma cell phenotype. Oral cavity PBL was diagnosed. Blood tests recorded mild lymphopenia and positive human immunodeficiency viruses serology.

Interventions: Patient underwent chemotherapy including intrathecal methotrexate prophylaxis, in addition to a highly active antiretroviral therapy.

Outcomes: At 12 months from diagnosis, patient recorded complete hematological remission.

Conclusions: Oral PBL diagnosis requires a high level of suspicion and awareness both by physicians and pathologists. They should be aware of the extent of such disease which is often mistaken as oral abscess or infected tooth, thus leading to delay the most appropriate diagnostic evaluation. As PBL is an aggressive non-Hodgkin lymphoma, a delayed diagnosis might negatively impact on both treatment and survival.
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http://dx.doi.org/10.1097/MD.0000000000022335DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7523795PMC
September 2020

Enasidenib and ivosidenib in AML.

Minerva Med 2020 Oct 21;111(5):411-426. Epub 2020 Sep 21.

Unit of Hematology, IRCCS Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST), Meldola, Forlì-Cesena, Italy.

The isocitrate dehydrogenases enzymes, IDH1 and IDH2, catalyze the conversion of isocitrate to α-ketoglutarate (αKG) in the cell cytoplasm and mitochondria, respectively, and contribute to generating the dihydronicotinamide-adenine dinucleotide phosphate (NADPH) as reductive potential in different cellular processes. Mutations in IDH1 and IDH2 genes are found collectively in about 20-25% of acute myeloid leukemia (AML) patients. Mutant IDH enzymes have neomorphic activity and convert αKG to the oncometabolite R-2-hydroxyglutarate (R-2-HG) which accumulates at high levels in the cell and hampers the function of αKG-dependent enzymes, including epigenetic regulators, thus leading to altered gene expression and block of differentiation and contributing to leukemia development. Inhibition of the neomorphic mutants induces marked decrease in R-2-HG levels and restores myeloid differentiation. Enasidenib and ivosidenib are potent and selective inhibitors of mutant IDH2 and IDH1, respectively, act as differentiating agents and showed clinical activity in relapsed/refractory (R/R) AML harboring the specific mutation. As single agents, both drugs have been approved by the Food and Drug Administration (FDA) for the treatment of R/R AML. The relevance of IDH targeting within either single agent approach or, most importantly, combinatorial treatments in AML will be discussed.
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http://dx.doi.org/10.23736/S0026-4806.20.07024-XDOI Listing
October 2020

Collision diffuse large B cell lymphoma and myeloid sarcoma in the liver.

Ann Hematol 2020 Aug 28. Epub 2020 Aug 28.

Institute of Hematology and Centre of Haemato-Oncology Research (CREO), University and Hospital of Perugia, Perugia, Italy.

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http://dx.doi.org/10.1007/s00277-020-04224-xDOI Listing
August 2020

Dutcher bodies in IgA multiple myeloma.

Ann Hematol 2020 Sep 23;99(9):2205-2206. Epub 2020 Jul 23.

Department of Experimental Medicine-Section of Pathologic Anatomy and Histology Medical School, University of Perugia, Perugia, Italy.

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http://dx.doi.org/10.1007/s00277-020-04192-2DOI Listing
September 2020

Bone marrow coexistence of chronic lymphocytic leukemia and Langerhans cell sarcoma.

Ann Hematol 2020 Dec 3;99(12):2957-2959. Epub 2020 Jul 3.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

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http://dx.doi.org/10.1007/s00277-020-04167-3DOI Listing
December 2020

Erythematous cutaneous macules: an uncommon, challenging presentation of T-lymphoblastic lymphoma.

Eur J Dermatol 2020 Jun;30(3):318-320

Pathology Unit, Azienda Ospedaliera S. Maria di Terni, University of Perugia, Terni.

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http://dx.doi.org/10.1684/ejd.2020.3772DOI Listing
June 2020

Prenatal diagnosis of familial hemophagocytic lymphohistiocytosis: morphological findings in the product of conception.

Ann Hematol 2021 Feb 4;100(2):585-586. Epub 2020 Jun 4.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

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http://dx.doi.org/10.1007/s00277-020-04108-0DOI Listing
February 2021

Huge mass of the scalp: Follicular lymphoma with complete regression after therapy.

Dermatol Ther 2020 07 28;33(4):e13608. Epub 2020 May 28.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

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http://dx.doi.org/10.1111/dth.13608DOI Listing
July 2020

Clinical, pathological and molecular features of plasmablastic lymphoma arising in the gastrointestinal tract: A review and reappraisal.

Pathol Res Pract 2020 Jun 25;216(6):152973. Epub 2020 Apr 25.

Pathology Unit, Azienda S. Maria di Terni, University of Perugia, Terni, Italy.

Plasmablastic lymphoma (PBL) is a CD20-negative large B-cell lymphoma with a plasmacytic phenotype and a dismal prognosis, which has been defined as a distinct entity only in the 2008 WHO Classification of Haematopoietic and Lymphoid Tissue and confirmed in the 2017 Edition. Current knowledge of the biological, clinical and prognostic features of PBL is mostly limited, resulting in diagnostic issues, as well as in lack of standard of care and effective therapeutic options. PBL commonly affects the oral cavity of HIV-positive individuals, however the gastrointestinal (GI) tract is the most common extraoral site, and in this location most patients are HIV-negative. In this review, we focus on the clinical, morphological and prognostic features of PBL arising in the GI tract, in order to improve knowledge on this rare, but aggressive disease.
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http://dx.doi.org/10.1016/j.prp.2020.152973DOI Listing
June 2020

Uterine myeloid sarcoma.

Ann Hematol 2020 Jul 4;99(7):1671-1672. Epub 2020 May 4.

Department of Experimental Medicine-Section of Pathologic Anatomy and Histology Medical School, University of Perugia, Perugia, Italy.

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http://dx.doi.org/10.1007/s00277-020-04065-8DOI Listing
July 2020

Germinotropic lymphoproliferative disorder: a systematic review.

Ann Hematol 2020 Oct 19;99(10):2243-2253. Epub 2020 Apr 19.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

Germinotropic lymphoproliferative disorder is a rare and rather enigmatic novel entity with distinctive clinicopathological features, one of which is the typical co-infection by Human herpesvirus 8 and Epstein-Barr virus. Human herpesvirus 8 is a lymphotropic virus detected in Kaposi sarcoma, multicentric Castleman disease, primary effusion lymphoma, Human herpesvirus 8-positive diffuse large B cell lymphoma not otherwise specified, and germinotropic lymphoproliferative disorder. Co-infection by Human herpesvirus 8 and Epstein-Barr virus is identified only in two lymphoproliferative diseases: germinotropic lymphoproliferative disorder and primary effusion lymphoma, which are otherwise diseases with totally different clinical presentations and outcomes. Unlike primary effusion lymphoma mostly occurring in immunocompromised individuals and following an aggressive course, germinotropic lymphoproliferative disorder usually presents with single or multiple lymphadenopathy affecting mainly immunocompetent individuals and mostly follows an indolent course. Based on the PRISMA guidelines, we carried out a systematic search on PubMed/MEDLINE, Web of Science, Scopus, EMBASE, and Cochrane Library using the search terms "germinotropic" and "lymphoproliferative disorder." Current scientific literature reports just 19 cases of germinotropic lymphoproliferative disorder. The purpose of our systematic review is to improve our understanding of the disease, focusing on epidemiology, clinical presentation, pathological features, treatment, and outcome. In addition, we discuss the differential diagnosis with the other Human herpesvirus 8-related lymphoproliferative diseases as currently recognized in the World Health Organization classification, adding a focus on lymphoproliferative disorders showing overlapping features.
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http://dx.doi.org/10.1007/s00277-020-04024-3DOI Listing
October 2020

Placental myeloid sarcoma.

Blood 2020 04;135(16):1410

University and Hospital of Perugia.

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http://dx.doi.org/10.1182/blood.2020004975DOI Listing
April 2020

Subcutaneous marginal zone lymphoma: an unusual lipoma-like presentation.

Ann Hematol 2020 Jun 7;99(6):1409-1410. Epub 2020 Apr 7.

Pathology Unit, Ospedale di Terni-University of Perugia, Terni, Italy.

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http://dx.doi.org/10.1007/s00277-020-04011-8DOI Listing
June 2020

The broad landscape of follicular lymphoma: Part II.

Pathologica 2020 Jun 12;112(2):79-92. Epub 2020 Mar 12.

Pathology Unit, Azienda Ospedaliera Santa Maria Terni, University of Perugia, Terni, Italy.

Follicular lymphoma is a neoplasm derived from follicle center B cells, typically both centrocytes and centroblasts, in variable proportions according to the lymphoma grading. The pattern of growth may be entirely follicular, follicular and diffuse and rarely completely diffuse. It represents the second most common non-Hodgkin lymphoma, after diffuse large B-cell lymphoma and it is the most common low-grade mature B-cell lymphoma in Western countries. In the majority of cases, follicular lymphoma is a nodal tumor, occurring in adults and is frequently associated with the translocation t(14;18)(q32;q21)/IGH-BCL2. However, in recent years the spectrum of follicular lymphoma has expanded and small subsets of follicular lymphoma, which differ from common follicular lymphoma, have been identified and included in the current 2017 WHO classification. The aim of our review is to describe the broad spectrum of follicular lymphoma, pointing out that the identification of distinct clinicopathological variants of follicular lymphoma is relevant for the patient outcomes and treatment.
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http://dx.doi.org/10.32074/1591-951X-6-20DOI Listing
June 2020

The broad landscape of follicular lymphoma: Part I.

Pathologica 2020 Mar 22;112(1):1-16. Epub 2020 Jan 22.

Pathology Unit, Azienda Ospedaliera Santa Maria Terni, University of Perugia, Terni, Italy.

Follicular lymphoma is a neoplasm derived from follicle center B cells, typically both centrocytes and centroblasts, in variable proportions according to the lymphoma grading. The pattern of growth may be entirely follicular, follicular and diffuse, and rarely completely diffuse. It represents the second most common non-Hodgkin lymphoma, after diffuse large B-cell lymphoma and is the most common low-grade mature B-cell lymphoma in western countries. In the majority of cases, follicular lymphoma is a nodal tumor, occurring in adults and frequently associated with the translocation t(14;18)(q32;q21)/IGH-BCL2. However, in recent years the spectrum of follicular lymphoma has expanded and small subsets of follicular lymphoma, which differ from common follicular lymphoma, have been identified and included in the current 2017 WHO classification. The aim of our review is to describe the broad spectrum of follicular lymphoma, pointing out that the identification of distinct clinicopathological variants of follicular lymphoma is relevant for patient outcomes and choice of treatment.
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http://dx.doi.org/10.32074/1591-951X-35-19DOI Listing
March 2020

An Italian Case of Disseminated Histoplasmosis Associated with HIV.

Case Rep Infect Dis 2019 16;2019:7403878. Epub 2019 Nov 16.

Infectious Diseases Clinic, Perugia University, S. Maria della Misericordia Hospital, Perugia 06132, Italy.

is a dimorphic fungus, endemic in the Americas, Africa (var. ), India, and Southeast Asia. infection is rarely diagnosed in Italy, while in Latin America, progressive disseminated histoplasmosis (PDH) is one of the most frequent AIDS-defining illnesses and causes of AIDS-related deaths. We report a case of PDH and new HIV infection diagnosis in a Cuban patient, who has been living in Italy for the past 10 years. Bone marrow aspirate and peripheral blood smear microscopy suggested infection. The diagnosis was confirmed with the culture method identifying its thermal dimorphism. Liposomal amphotericin B was administered alone for 10 days and then for another 2 days, accompanied with voriconazole; the former was stopped for probable side effects (persistent fever and worsening thrombocytopenia), and voriconazole was continued to complete 4 weeks. PDH maintenance treatment consisted of itraconazole for one year. Antiretroviral therapy (ART) was started on the third week of antifungal treatment. At the 3-year follow-up, the patient is adherent on ART, the virus was suppressed, and she has an optimal immune recovery. This case highlights the need to suspect histoplasmosis in the differential diagnosis of opportunistic infections in immunocompromised persons, native to or who have traveled to endemic countries.
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http://dx.doi.org/10.1155/2019/7403878DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6885199PMC
November 2019

Atypical mycobacteria diagnosed by bone marrow biopsy in an HIV-positive patient.

Ann Hematol 2020 Jan 6;99(1):183-184. Epub 2019 Dec 6.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

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http://dx.doi.org/10.1007/s00277-019-03880-yDOI Listing
January 2020

A tricky and rare cause of pulmonary eosinophilia: myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA.

BMC Pulm Med 2019 Nov 19;19(1):216. Epub 2019 Nov 19.

Pathology Unit, Ospedale di Terni, University of Perugia, Perugia, Italy.

Background: Eosinophilic lung diseases represent a heterogeneous group of disorders with prominent infiltrate of eosinophils in lung interstitium and alveolar spaces. Peripheral blood eosinophilia is often present. Infections, drugs, allergens, toxic agents have to be evaluated as possible causes of eosinophilic lung infiltrates. The category of myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, FGFR1 and PCM1-JAK2 represents an uncommon cause of eosinophilic lung infiltrate.

Case Presentation: We report the case of a 70-year old man complaining of dry cough and dyspnea. Ground glass-opacities were seen on imaging studies and peripheral blood eosinophilia was present. A thorough step-wise patient's evaluation led to identify the clonal nature of eosinophilia and the diagnosis of myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA was made.

Conclusions: Correlation with clinical history, laboratory tests and imaging studies is essential to achieve the correct diagnosis when facing with eosinophilic lung infiltrates. A prolonged eosinophilia can cause life-threatening organ damage. Identification of PDGFRA rearrangement, as in the present case, is particularly critical given the sensitivity and excellent response to imatinib, which has completely changed the natural history of this neoplasm.
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http://dx.doi.org/10.1186/s12890-019-0967-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6862820PMC
November 2019

Fibrin-associated large B-cell lymphoma: first case report within a cerebral artery aneurysm and literature review.

BMC Cancer 2019 Sep 13;19(1):916. Epub 2019 Sep 13.

Pathology Unit, Ospedale di Terni, University of Perugia, Perugia, Italy.

Background: Fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is a rare Epstein-Barr virus (EBV) positive lymphoproliferative disorder included in the current World Health Organization (WHO) classification. It arises within fibrinous material in the context of hematomas, pseudocysts, cardiac myxoma or in relation with prosthetic devices. In these clinical settings the diagnosis requires an high index of suspicion, because it does not form a mass itself, being composed of small foci of neoplastic cells. Despite overlapping features with diffuse large B-cell lymphoma associated with chronic inflammation, it deserves a separate classification, being not mass-forming and often following an indolent course.

Case Presentation: A 64-year-old immunocompetent woman required medical care for cerebral hemorrhage. Computed Tomography (CT) angiography identified an aneurysm in the left middle cerebral artery. A FA-DLBCL was incidentally identified within thrombotic material in the context of the arterial aneurysm. After surgical removal, it followed a benign course with no further treatment.

Conclusions: The current case represents the first report of FA-DLBCL identified in a cerebral artery aneurysm, expanding the clinicopathologic spectrum of this rare entity. A complete literature review is additionally made.
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http://dx.doi.org/10.1186/s12885-019-6123-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6743119PMC
September 2019

Extracavitary primary effusion lymphoma in a post-transplantation patient.

Br J Haematol 2019 12 13;187(5):555. Epub 2019 Sep 13.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

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http://dx.doi.org/10.1111/bjh.16196DOI Listing
December 2019

HHV-8- and EBV-positive germinotropic lymphoproliferative disorder.

Ann Hematol 2019 Oct 3;98(10):2439-2441. Epub 2019 Aug 3.

Pathology Unit, Azienda Ospedaliera S. Maria di Terni, University of Perugia, Perugia, Italy.

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http://dx.doi.org/10.1007/s00277-019-03773-0DOI Listing
October 2019