Hematopathology Diagnosis

$1,099,671ZIAFY2021CANIH

Division Of Basic Sciences - Nci

Investigators

Linked publications & trials

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Abstract

Dr. Pittaluga and I aid in the diagnosis and classification of reactive and neoplastic lymphoproliferative disorders, immunodeficiency states, and diverse hematological malignancies. We provide consultative and collaborative services to physicians in the NCI, as well as to physicians studying patients with hematolymphoid disorders in other institutes, in particular NIAID, NHLBI, NHGRI, and NIAMSD. We regularly present at conferences sponsored by clinical branches in the NCI and other Institutes (NIAID, NHLBI, and NHGRI.) We receive more than 2000 cases in consultation each year. We try to restrict consultations to difficult or challenging cases, and do not accept cases for routine second opinions. A significant proportion (approximately 20%) of the cases are submitted by other academic institutions based on diagnostic uncertainty, or because of differences of opinion among several institutions. We are often the final arbiter on challenging diagnostic problems. Additionally, we have made novel observations based on our clinical practice, and a number of publications have emanated from case material originally reviewed in consultation. Our clinical consultation practice also synergizes with other NIH clinical groups, enhancing referral to NIH clinical protocols. Our work has led to improvement in the diagnosis and classification of several rare diseases including multicentric Castleman disease, KSHV- and EBV-associated proliferations, and aggressive B-cell lymphomas, including double-hit and triple-hit lymphomas with CCND1 translocation. The term grey zone lymphoma has been used for lymphomas with hybrid morphological and phenotypic features between two well defined entities, particularly classic Hodgkin lymphoma and diffuse large B-cell lymphoma, but also for lymphomas with intermediate features between nodular lymphocyte predominant Hodgkin lymphoma and T-cell/histiocytic-rich large B-cell lymphoma or EBV-positive classic Hodgkin lymphoma and EBV-positive diffuse large B-cell among others. We have clarified the diagnostic criteria for the diagnosis of grey zone lymphoma, and the interrelationship between classic Hodgkin lymphoma and other B-cell neoplasms.

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