Hematology: Ch. 34 Mature Lymphoid Neoplasms

What are Mature Lymphoid Neoplasms?

Diseases caused by malignant transformation of normal lymphoid cells affecting the lymph nodes, spleen, or other extra-nodal sites. Result in abnormal growths.

Primary site of Leukemias?

Primary site of the disease is the blood or bone marrow.

Characteristic's of Leukemia?

~Begins in the bone marrow

~Abnormally high production of immature and abnormally functioning leukocytes.

~Spreads through the bloodstream

Primary site of Lymphomas?

Primary site of the disease is in the lymph nodes or other lymphatic tissue.

Characteristics of Lymphomas?

~Begins in the lymphatic system

~Spreads through lymph nodes

~WBC count may be low or high, and rarely have immature cells

The WHO 2022 has ____ main categories of lymphomas.

Three

The main categories created by WHO for classification of lymphoid neoplasms are?

~Mature B-cell neoplasms

~Plasma cell neoplasms and other diseases with paraprotein

~Mature T-cell and NK-cell neoplasms

What factors are used for neoplasm diagnosis?

~Clinical features

~Morphological features

~Immunophenotyping

~Genetic findings

Presence of monoclonal lymphocytes in peripheral blood, but not enough to make a diagnosis of CLL. Affects women over the age of 40.

Monoclonal B-cell lymphocytosis

Risk of progression from MBL to CLL is ___ to ___ per year.

1% to 2%

How is MBL detected?

Detected through flow cytometry

What is the most common leukemia in adults?

Chronic Lymphocytic Leukemia (CLL)

Bone marrow is replaced by well-differentiated lymphocytes with cobblestone or soccer ball appearance. This type of leukemia is?

CLL

CLL presents as accumulation of mature appearing functionally incompetent B lymphocytes that (abnormally) express ___.

CD5

_____ lymphocytes are a common finding in CLL, count as lymphocytes but only in diagnosed CLL patients or when using albuminized film.

Smudge

Slow disease of B cell linage primarily presenting splenomegaly with cytopenia's and Hairy cells found in peripheral blood and bone marrow aspirate. Marrow fibrosis is present.

Hairy Cell Leukemia

Cell with kidney-bean shaped nuclei and ragged hair-like projections around entire cell.

Hairy cells

What is used to detect HCL?

Immunochemistry with anti-CD20 antibody can help identify clusters of hairy cells.

This cellular protein is specific to HCL.

Annexin A

Rare disease characterized by greater than 15% lymphocytes being large granular lymphs.

Large Granular Lymphocytic Leukemia

This cell has abundant blue cytoplasm with distinct medium to large azurophilic cytoplasmic granules.

Large granular lymphocytes

What are the two types of LGL classifications under WHO 2022?

T-LGL and NK-LGL

T-LGL T-cells express?

CD3, CD8, CD57 with TDR gene

NK-LGL are negative negative for ___ but express _____.

CD3; CD56

What disease is associated with retroviral infection by human T-lymphotropic virus type 1?

Adult T cell Leukemia/Lymphoma (ATLL)

What type of involvement is extensive in Adult T cell Leukemia/Lymphoma (ATLL)?

Extranodal involvement

What type of cells are present in Adult T cell Leukemia/Lymphoma (ATLL)?

Flower cells

What is Burkitt lymphoma/leukemia (BL)?

Aggressive cancer of mature B cells with sudden onset and rapid tumor growth.

In which region is the endemic form of Burkitt lymphoma commonly found?

Equatorial Africa during childhood.

What distinct feature do Burkitt lymphoma cells have?

Distinct vacuoles.

What are the three subtypes of Burkitt lymphoma?

Endemic, sporadic, and immunodeficiency-associated.

Neoplastic disorder of germinal B cells and the second most common type of non-Hodgkin lymphoma (NHL).

Follicular lymphoma (FL)

What percentage of non-Hodgkin lymphoma (NHL) does follicular lymphoma represent?

About 12%

What are the common symptoms of follicular lymphoma?

Painless lymphadenopathy and asymptomatic

What distinct feature do B cells have in follicular lymphoma?

Distinct nuclear clefts

Patients with this disease present with extensive lymphadenopathy, and the GI tract is the primary area of involvement.

Mantle Cell Lymphoma (MCL)

What is required for Diagnosis of MCL?

Diagnosis requires demonstration of t(11,14) via FISH or overexpress of cyclin D1 by immunochemistry.

This disease is most the most common form of NHL can result as a transformation from another lymphoma, and presents rapidly expanding, but painless, lymphadenopathy's in one or more sites.

Diffuse large B-cell Lymphoma (DLBCL)

Cells in DLBCL present as?

Large cells with moderate amounts of basophilic cytoplasm, and several round/irregular shaped nuclei.

Slow progress B cell lymphoma associated with chronic antigen stimulation in infection or autoimmunity. Patients present with massive splenomegaly and absolute lymphocytosis with the presence of villous lymphocytes (often confused for hairy cells).

Marginal Zone Lymphoma (MZL)

What are three subtypes for MZL and which one is the most common?

Extranodal marginal zone of lymphoma of mucosa-associated lymphoid tissue (MALT) is most common

Splenic marginal zone lymphoma

Nodal marginal zone lymphoma

How do cells appear when you have MZL?

Cells have cytoplasmic projections with a polar distribution (rather than circumferential projections seen in hairy cells)

What is the most common form of cutaneous T cell lymphoma also regarded as a slow disease?

Mycosis fungoides (MF) (accounting for 60-70% of cases)

Sezary Syndrome (SS) is _____ with peripheral blood involvement and worse prognosis than MF.

Systemic

These cells have abnormal appearance with scant cytoplasm and a cerebriform, folded nucleus, variably condensed chromatin, and inconspicuous nucleoli.

MF and SS cells

This disease is a T-cell disorder with large pleomorphic cells that have irregular lobulated nuclei resembling blasts.

Anaplastic Large Cell Lymphoma (ALCL)

ALCL tumors express _____

CD30

ALCL + ____ accounts for 10-30% of childhood leukemias. ___ however is more common in older patients and has been associated with breast implants.

ALK

Abnormal cells characterized by T-cell antigen mismatch (absence of CD7 or CD5 expression) and are larger, basophilic with pleomorphic appearance.

Peripheral T-cell lymphoma, Not otherwise Specified (PTL-NOS)

PTL-NOS is often present with _______ _________.

Disseminated disease

This disease makes up 95% of Hodgkin Lymphoma cases and presents with cervical, supraclavicular, and mediastinal adenopathy that spreads in a contiguous manner.

Classic Hodgkin Lymphoma (CHL)

These cells are found in Classic Hodgkin Lymphoma (CHL).

Reed-Sternberg cells

Reed-Sternberg cells primarily express ____ and co-express ____.

CD30 and CD15

This disease is mainly seen in young adults, lacks Reed-Sternberg cells, and presents with peripheral lymphadenopathy (neck, armpits, groin).

Nodular Lymphocyte Predominant HL (NLPHL)

What malignant cells that express epithelial membrane antigen (EMA) are seen in NLPHL?

L&H cells

L&H cells are also called _______ ; what kind of growth pattern do they have?

Popcorn cells; Scant cytoplasm and a folded single nucleus

What is the difference between Reed-Sternberg and L&H cells?

L&H cells lack CD15; CD30 expression

_____ are malignant disorders of terminally differentiated B cells and is the 2nd most common hematological malignancy AFTER CLL.

Plasma Cell Neoplasm (PCNs)

What is the precursor state of Multiple Myeloma?

Monoclonal gammopathy of unknown significance (MGUS)

Bone marrow-based disease with extramedullary extension (soft bone and tissue) with strict criteria for diagnosis known as CRAB.

Multiple Myeloma

How does Multiple Myeloma present?

Primary plasma cell leukemia and progression to secondary plasma cell leukemia are marked by the presence of 5% or more plasma cells on the peripheral blood film.

Presence of ______ in MM is evidence for a circulating monoclonal protein.

Rouleaux

Malignant plasma cells in MM express high levels of _____ and ____ and low levels of ______.

CD38 and CD138; CD45

What is the localized form of PCN?

Solitary Plasmacytoma

Heavy chain disease characterized by low-grade lymphoplasmacytic lymphoma (LPL) with aberrant secretion of IgM, resulting in hyperviscosity syndrome.

Waldenstrom Macroglobinuria

This factor is a distinct somatic mutation found in 90% of patients with Waldenstroms.

Myeloid differentiation factor 88 (MY88)

How does the peripheral blood appear in Waldenstroms?

Rouleaux, increase in small lymphocytes and/or plasma cells

This staging system is used for Hodgkin Lymphoma, quantifies nodal and extranodal sites of involvement, and is used to define limited versus extensive disease, but is less useful in NHL.

Ann Arbor Staging System

This classification system is a simplified version of Ann Arbor used for NHLs and groups patients into limited (stages 1&2) versus advanced (stages3&4)

Lugano Classification

____ has largely supplanted anatomic staging as the primary prognostic tool for NHL. Patients are grouped into 4 prognostic groups, with group 1 being low risk to group 4 being high risk.

Internal Prognostic Index (IPI)