Lymphomas & Plasma Cell Dyscrasias Flashcards by Maggie Gilman

What are lymphomas?

malignant neoplasms of cells native to lymphoid tissue/nodes and most often of B-cell origin

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What are the two primary forms of lymphoma?

Non-Hodgkin lymphoma (NHL); Hodgkin lymphoma (HL)

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what are general features of all lymphomas?

either NO symptoms OR any combo of:
- lymphadenopathy (silent)
- high WBC count
- infection
- weight loss
- fever
- malaise

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Describe the clinical presentation of Non-Hodgkin’s lymphoma (NHL)

lymphadenopathy (without tenderness/pain)
potential spread from single node to others (non-contiguous), or to non-nodal tissue (spleen, liver, bone marrow)
potential to “spill” into blood

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What are characteristics of healthy lymph nodes?

Mobile, soft, nontender and less than 1 cm

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What method is used to classify all types of lymphomas?

Revised European-American Classification of Lymphoid Neoplasms (REAL)

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What are examples of adult forms of Non-Hodgkin’s lymphoma (NHL)?

Follicular lymphoma
Small lymphocytic lymphoma
Large B-Cell lymphoma

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What form of Non-Hodgkin’s lymphoma can potentially progress to Large B-Cell Lymphoma?

Follicular lymphoma

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What will be the histological presentation of the lymph nodes of a patient with follicular lymphoma?

Nodular (follicular) lymph nodes

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What is the most aggressive and rapidly fatal form of adult Non-Hodgkin’s Lymphoma (NHL)?

Large B-Cell lymphoma

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What will be the histological presentation of the lymph nodes of a patient with Large B-Cell Lymphoma?

Diffuse lymph nodes

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What form of adult Non-Hodgkin’s Lymphoma (NHL) involves rapid metastasis to the brain, bone, and GI?

Large B-Cell lymphoma

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What is the primary way to differentiate between small lymphocytic lymphoma and chronic lymphocytic leukemia upon palpation?

Small lymphocytic lymphoma = Nodal origin with lump present
Chronic lymphocytic leukemia = Bone pains, marrow origin

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What are examples of childhood Non-Hodgkin’s Lymphoma’s (NHL)?

Acute lymphoblastic lymphoma
Burkitt’s lymphoma

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Where is a mass most common in a patient with acute lymphoblastic leukemia?

Mediastinum

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What is the histological presentation indicative of any childhood Non-Hodgkin’s lymphoma?

Starry Sky due to macrophages present

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what is the prognosis of acute lymphoblastic lymphoma?

aggressive and rapidly progressive

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Male patient. 11 years of age. Presents with chronic dry cough; loss of appetite, and achy pain in the left substernal region. Biopsy of the mediastinal lymph nodes shows the presence of macrophages giving a “Starry” appearance to the slide. What is a likely diagnosis?

Acute lymphoblastic lymphoma

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Male patient. 30 years of age. Presents with swollen, non-tender cervical lymph nodes, fever, and malaise. Since the lymph nodes are not mobile upon palpation, a biopsy of the tissue is taken. Biopsy shows nodular growths throughout the lymph nodes. What is a likely diagnosis?

Follicular lymphoma

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Male patient. 37 years of age. Presents with systemic infections, high-grade fever, and a WBC count of 80,000. The patient reports of constipation and headaches for the last three days. Upon inspection, lymphadenopathy is present in the axillary and deep cervical lymph nodes. The nodes are not tender and measure 2 cm. A biopsy of the nodes is taken. Biopsy shows diffuse growth of lymph nodes in both the cervical and axillary regions. What is a likely diagnosis?

Large B-cell lymphoma

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What nationality is Burkitt’s lymphoma endemic to?

African

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What nodal area is primarily affected in a patient with Burkitt’s lymphoma?

Maxilla/Mandible

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What virus is associated with the African form of Burkitt’s lymphoma?

Epstein-Barr Virus (HHV-4)

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Epstein-Barr virus is associated with the sporadic form of Burkitt’s lymphoma (TRUE/FALSE)?

FALSE

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What form of Non-Hodgkin's lymphoma (NHL) can present with craniofascial defects?

Burkitt's lymphoma

what is the prognosis of Burkitt's lymphoma?

responds well to chemo

What is the most common malignancy in young adults?

Hodgkin's lymphoma

What special lymphocyte is present in most cases of Hodgkin's lymphoma?

Reed-Sternberg (RS) cell

What are the histological characteristics of the Reed-Sternburg cell?

- large cells (possibly B or T cell origin) - Bilobed/Binucleated - "Owl-Eyes" appearance

What are the main characteristics that differentiate Hodgkin's lymphoma from Non-Hodgkin's lymphoma?

Hodgkin's is predictable and contiguous (adjacent) spread of lymph nodes

What are the forms of Hodgkin's Lymphoma?

- Nodular-Sclerosis Hodgkin Lymphoma (NSHL) - Mixed-Cellularity Hodgkin Lymphoma (MCHL) - Lymphocyte-Rich Hodgkin Lymphoma (LRHL) - Lymphocyte-Depleted Hodgkin Lymphoma (LDHL)

Hodgkin's lymphoma primarily occurs in (Young adults/Elderly)?

Young adults

Describe the symptomology of Hodgkin's lymphoma

non-specific

What is the most common form of Hodgkin's lymphoma (HL)?

Nodular-Sclerosis Hodgkin Lymphoma (NSHL)

what is the second most common form of Hodgkin's Lymphoma?

Mixed-Cellularity Hodgkin Lymphoma (MCHL)

What histological evidence is present in a patient with Nodular-Sclerosis Hodgkin Lymphoma (NSHL)?

Lacunar (clearing around) RS cell with collagen bindings present

What is the most common anatomical site of lymph involvement in a patient with Nodular-Sclerosis Hodgkin Lymphoma (NSHL)?

Mediastinum

What lymph nodules are most commonly affected in a patient with Nodular-Sclerosis Hodgkin Lymphoma (NSHL)?

Lower cervical, mediastinal, and supraclavicular lymph nodes

what is the nodal presentation of a patient with Nodular-Sclerosis Hodgkin Lymphoma?

nodular architecture: lymphoid tissue surrounded by fibrosis

Female patient. 20 years of age. Presenting with lymphadenopathy and fatigue. Palpable lumps are present near her mediastinal and supraclavicular regions. The lumps are not tender. Biopsy is taken on the supraclavicular and mediastinal lymph nodes. Large, Bi-lobed lacunar cells are present with collagen deposition in the nodes. What is the likely diagnosis? What is the name given to the bi-lobed cells described?

- Nodular-Sclerosis Hodgkin Lymphoma (NSHL) - Reed Sternburg cell

What cells are present in higher numbers in a patient with Mixed-Cellularity Hodgkin Lymphoma (MCHL)?

Eosinophils, neutrophils, macrophages, and plasma cells

What is the histological presentation of a patient with Mixed-Cellularity Hodgkin Lymphoma (MCHL)?

Typical Reed Sternberg cells with NO COLLAGEN bands

What is the nodal presentation of a patient with Mixed-Cellularity Hodgkin Lymphoma (MCHL)?

Diffuse lymph nodes

What forms of Hodgkin's Lymphoma (HL) are associated with an HIV infection?

Mixed-Cellularity Hodgkin Lymphoma (MCHL) (HIV-1) Lymphocyte-Depleted Hodgkin Lymphoma (LDHL)

Male patient. 28 years of age. Patient history shows diagnosis of HIV-1 at age 22. Patient presents today with a fever, fatigue, malaise, and swollen axillary lymph nodes. Recent CBC testing shows polymorphonucleocytosis, eosinophilic leukocytosis, and monocytosis. A biopsy is taken on the axillary lymph nodes. Nodal presentation is diffuse and Reed Sternburg cells are present with the absence of collagen. What is a likely diagnosis?

Mixed-Cellularity Hodgkin Lymphoma (MCHL)

What is the nodal presentation of a patient with Lymphocyte-Rich Hodgkin Lymphoma (LRHL)?

Nodular (follicular) lymph nodes

What cells will be present in high amounts in a patient with Lymphocyte-Rich Hodgkin Lymphoma (LRHL)? Low amounts?

High amounts of lymphocytes and macrophages and low amounts of Reed Sternburg cells

Which form of Hodgkin's Lymphoma (HL) presents with a very low Reed Sternburg cell count?

Lymphocyte-Rich Hodgkin's Lymphoma( LRHL)

Male patient. Age 24. Presenting with lymphadenopathy, fever, and malaise. Lymph nodes in the deep cervical and supraclavicular region are swollen and non-mobile measuring approximately 1.62 cm. A biopsy is taken showing large amounts of mature lymphocytes and macrophages, with low amounts of Reed Sternburg cells present. The nodal presentation shows follicular growths throughout. What is a likely diagnosis?

Lymphocyte-Rich Hodgkin Lymphoma (LRHL)

What cells will be present in high amounts in a patient with Lymphocyte-Depleted Hodgkin Lymphoma (LDHL)? Low amounts?

High amounts of Reed Sternburg cells and low amounts of lymphocytes

What form of Hodgkin's lymphoma is correlated with both an HIV and an EBV infection?

Lymphocyte-Depleted Hodgkin Lymphoma (LDHL)

Male patient aged 18 presents with systemic infections and fevers. Patient history shows HIV inheritance passed on from the patients mother at birth as well as an infectious mononucleosis infection in the past year. Patient is experiencing lymphadenopathy in their axillary and supraclavicular lymph nodes. A biopsy is taken and shows high amounts of Reed Sternburg cells and low amounts of lymphocytes. What is a likely diagnosis?

Lymphocyte-Depleted Hodgkin Lymphoma (LDHL)

which form of Hodgkin's lymphoma has the worst prognosis without treatment?

lymphocyte depleted HL (followed by Nodular-Sclerosis HL)

What is the criteria for a stage I lymphoma?

Involvement of a single lymph node region

What is the criteria for a stage II lymphoma?

Involvement of two or more lymph node regions on the **same side** of the diaphragm

What is the criteria for a stage III lymphoma?

Involvement of two or more lymph node regions on **both sides** of the diaphragm

What is the criteria for a stage IV lymphoma?

Diffuse spread out of the lymph into non-lymph organs

Patient is diagnosed with Nodular-Sclerosing Hodgkin's Lymphoma (NSHL) in his axillary and supraclavicular lymph nodes. What stage is given to categorize his lymphoma?

Stage II

Patient is diagnosed with Lymphocyte-Rich Hodgkin's Lymphoma (LRHL) in his deep cervical lymph nodes. What stage is given to categorize his lymphoma?

Stage I

Patient is diagnosed with Mixed-Cellularity Hodgkin's Lymphoma (MCHL) in his deep cervical and popliteal lymph nodes. What stage is given to categorize his lymphoma?

Stage III

Patient who is diagnosed with Lymphocyte-Deficient Hodgkin's Lymphoma (LDHL) has experienced the spread of his axillary and inguinal lymph nodes to the skeletal muscle tissue in his breast and right thigh. What stage is given to categorize this lymphoma?

Stage IV

where would you expect a case of Hodgkin's lymphoma to metastasize to?

from lymph nodes -> spleen -> liver

What is an example of a plasma cell dyscrasia?

Multiple myeloma

Multiple myeloma is (Benign/Malignant)?

Malignant

What population does multiple myeloma primarily affect?

Older individuals

What is multiple myeloma?

- primary bone neoplasm - malignancy of plasma cells

What is evident in a patient's blood if they have multiple myeloma?

Myeloma protein (M spike) Increased IgG (Bence-Jones protein) Hypercalcemia

what do malignant plasma cells secrete?

antibodies: myeloma protein (M spike)

what is an M spike?

monoclonal spike: mostly IgG Ab secreted by malignant plasma cells

What protein is seen in the urine in high amounts with patients who have multiple myeloma?

Bence-Jones protein

what is responsible for proteinuria seen in patients with multiple myeloma?

- large amounts of IgG dissociate into light chain protein fragments - IgG light chain damages kidney - proteins are spilled/excreted in urine

what is Bence-Jones protein?

light chain of IgG that damages kidney

what is the role of cytokines in multiple myeloma?

osteoclast activity leads to demineralization of bone to make room for plasma cell proliferation

What cytokine is implicated in the "Punched Out" lesions and "Raindrop skull" presentation in patients with multiple myeloma?

Osteoclast activating factor (OAF)

A patient with multiple myeloma will be (Hypocalcemic/Hypercalcemic)? Why?

Hypercalcemic due to demineralization of bones

What are clinical signs and symptoms of multiple myeloma?

Systemic infection Hypercalcemia Pathological fractures Proteinuria Renal failure/disease

What parts of the skeleton are involved in multiple myeloma?

Flat bones & irregular bones: Vertebrae Skull Long bones