W4-LP-LYMPHOID NEOPLASM Flashcards

Question

Outline the features of Primary extranodal lymphomas

Answer 1

- Briefly, this is a distinct group of tumours, predominantly of B cell phenotype. - Common sites of extranodal lymphoma, in descending order of frequency are the GIT, skin, Waldeyer’s ring, salivary glands, thymus gland, ocular adnexa, thyroid gland, lung and testis

Answer 2

- Adult T cell lymphoma/leukaemia - Peripheral T cell lymphoma, unspecified - Anaplastic large cell lymphoma - Mycosis fungoides/Sezary syndrome

Answer 3

- Confined to patients infected with the HTLV-1 retrovirus - Caribbean. - This is a disease of adults - The lymph node involved is diffusely replaced by neoplastic T cells, which vary in cell size and regularity. Neoplastic cells may also be seen in peripheral blood. - Patients often have aggressive disease, associated with lytic bone lesions and hypercalcaemia.

Answer 4

-This is also a disease of adults. - It has an aggressive course, but is potentially curable. - These neoplasms typically contain a mixture of small and large neoplastic T cells, often with irregular nuclei. - There may be marked infiltration of non-neoplastic cells in the background, including macrophages and eosinophil

Answer 5

- The majority of cases are children or young adults - The clinical pattern is variable, some showing widespread involvement of lymph nodes and other sites, and others being confined to skin. - The neoplastic cells tend to be larger than in any other type of lymphoma, with bizarre large cells, some multinucleated. - In most cases, cell lineage markers indicate a T cell phenotype. - A t(2;5) chromosomal translocation, causing fusion of ALK and NMP genes, may be demonstrated, detectable by immunostaining for ALK protein. - The tumours with this translocation tend to have a better prognosis.

Answer 6

- Mycosis fungoides is a T cell lymphoma seen most commonly in the skin, but which is referred to as Sezary syndrome when the characteristic cells are also found in the peripheral circulation. - The neoplastic cells accumulate in the epidermis, where they may form localized pockets referred to as “Pautrier’s micro-abscesses”, and have typical convoluted cerebriform nuclear morphology. As the disease progresses it can spread to lymph nodes, where the interfollicular zones are infiltrated. - The cells express T cell markers and are almost always of CD4 helper subtype.

Answer 7

- The age of presentation varies in different socioeconomic groups and countries. - The USA and Britain, see a bimodal age curve, with the first peak occurring between 15-35 years of age and the second peak beyond 50 years of age. - In underdeveloped countries, the disease is much more frequent under the age of 10 years. - Overall, males and females are equally affected. Nodular sclerosing HL affects women more frequently

Answer 8

Reed- Steenburg ccells

Answer 9

Hodkin's lymphoma *The neoplastic cells usually make up a small fraction of the total cellular component.

Answer 10

- HL starts as unifocal or unicentric disease. - In the vast majority of patients, HL is initially confined or localized to the superficial lymph nodes (particularly cervical lymph nodes). - There are well -defined, orderly and predictable patterns of spread in most patients. HL usually spreads to contiguous sites (areas or tissues that are next to or touching each other)

Answer 11

- Nodular sclerosis Hodgkin lymphoma * Lymphocyte-rich classic Hodgkin lymphoma * Mixed cellularity Hodgkin lymphoma * Lymphocyte depletion Hodgkin lymphoma ∗ Nodular lymphocyte predominance Hodgkin lymphoma

Answer 12

- most common type occurring in females - The neoplasm involves the mediastinum more commonly than all other types combined. - A very high incidence of contiguous spread is noted; i.e. occurring in more than 90% of cases. There are characteristic hallmarks of NS subtype: 1. The lymph node capsule shows fibrous thickening, from which broad bands extend into the parenchyma of the node, subdividing it into nodules. 2. A distinctive variant of the Reed-Sternberg cell, termed the “lacunar” cell occurs. 3. The cell cytoplasm shrinks and the nucleus appears to lie in a clear space

Answer 13

- The lymph node contains a mixed infiltrate of abnormal and reactive cells. - Diagnostic Reed-Sternberg cells are fairly easily found, as are mononuclear variants with prominent nucleoli. - Patients with this subtype often present with lymphadenopathy in two or more sites and systemic symptoms. This is considered a poor prognostic group.

Answer 14

- This subtype shows depletion of lymphocytes and numerous, often bizarre Reed- Sternberg cells. - Patients frequently have systemic “B” symptoms of fever and weight loss, as well as pancytopenia

Answer 15

- The reverse pattern of lymphocyte depletion, with abundant lymphocytes and scarce Reed-Sternberg cells, is seen.

Answer 16

- Scattered neoplastic cells are seen against a background of an abnormal cellular infiltrate. - Most of the neoplastic cells, however, known as “L & H” (lymphocytic and histiocytic) or “popcorn” cells, lie within large nodular areas made up of small lymphoid cells. - Molecular studies have shown that the neoplastic cells in this subtype are of B cell phenotype, different from that of classic Reed-Sternberg cells. - This subtype of HL shows a striking male predominance and its pattern of spread differs from that of the other HL subtypes. - It tends to be an indolent, slowly progressive disease, although occasional cases transform after a period into large cell lymphoma, usually of B cell type.

Answer 17

1. Stage of disease (assessed clinically and pathologically). 2. The presence or absence of B symptoms (indicative of advanced disease) 3. Histologic subtype – lymphocyte predominant most favourable and lymphocyte depleted disease, the worst subtype 4. Age – prognosis worsens with increasing age, especially over 60 years

Answer 18

1. Irradiation (usually for localized disease). 2. Multi-agent chemotherapy, given in disseminated disease.