- Not curable with chemo - May be cured with allogeneic SCT (rarely used d/t morbidity)

- MC adult lymphoma - Aggressive, frequently involves extra-nodal sites - CD 19 and 20 positive

Myeloma and Lymphomas Flashcards by Megan MacNabb

What is the origin of most lymphomas?

B cell

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What do B cells produce?

Immunoglobulin antibodies

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What do T cells produce?

Cytokines

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Which types of NHL are typically more aggressive but sometimes curable?

Larger cells

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Which types of NHL are typically less aggressive but generally incurable?

Smaller cells

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How does lymphadenopathy progress in NHL?

Spreads non-contiguously from peripheral LN groups inward toward central LN groups

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How does lymphadenopathy progress in Hodgkin’s lymphoma?

Spreads contiguously from central LN groups outward

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Describe the diagnostic lymph node in NHL

Most of the cells are malignant

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Describe the diagnostic lymph node in Hodgkin’s lymphoma

Only a minority of the cells are malignant (Reed-Sternberg)
Majority of cells are inflammatory and benign

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What are Reed-Sternberg cells?

Malignant cells in a lymph node of Hodgkin’s lymphoma
“Owl eyed” appearance
CD 30 positive

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MC adult leukemia?

CLL/SLL

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Describe CLL/SLL

Identical - differ only in how they are initially diagnosed (SLL if found in a lymph node)

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Typical lab findings of CLL

Absolute lymphocyte count over 5000 consisting of small mature B cells
CD 19 and 20 positive
CD 5 positive

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Treatment of CLL/SLL

Not curable with chemo

- May be cured with allogeneic SCT (rarely used d/t morbidity)

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What are CLL-like lymphoid leukemias?

Hairy cell leukemia

- Splenic marginal zone lymphoma/leukemia

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Classic lab finding of hairy cell leukemia

Small lymphocytes w/hairy projections

- CD 19, 20, 11c, 103 positive

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Treatment of hairy cell leukemia

Purine analogs (80% CR, 60% long term CR)

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Gastric MALTs are frequently caused by what?

H pylori infections

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Describe MALT lymphomas

Low grade lymphomas usually of lymphoid tissue a/w exocrine glandular tissue

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What can cure some early stage MALT lymphomas?

Treatment of H pylori infections

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What are follicular lymphomas characterized by?

Lymphocytes and CD 10, 20

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MC adult lymphoma?

DLBCL

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Describe DLBCL

MC adult lymphoma
Aggressive, frequently involves extra-nodal sites
CD 19 and 20 positive

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Standard treatment of DLBCL?

R-CHOP

Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone

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Describe Burkitt's lymphoma

- Highly aggressive - Endemic in sub-Saharan Africa - A/w EBV infection - Presents usually as a jaw mass (in Africa) or intra-abdominal mass (in US)

Prognosis of Burkitt's lymphoma

90% CR | 75% cure (long term CR)

Describe mantle cell lymphoma

Histologically low grade, but aggressive and incurable

How does mantle cell lymphoma typically present?

Bowel obstruction or intra-abdominal mass

Typical cytological findings of mantle cell lymphoma

- CD20 and 5 positive | - Cyclin D1 overexpressed

Median survival of mantle cell lymphoma

3.5 to 5 years

What do T cell lymphomas typically involve?

Skin and subcutaneous tissue

MC T cell lymphoma?

PTCL NOS | Peripheral T cell lymphoma not otherwise specified

How do PTCL NOS cases present?

- Nodal and skin involvement - Stage IV - CD4 and 5 positive

Treatment of PTCL NOS

CHOP (but only 56% CR, 36% 5 yr overall survival)

What is Mycosis Fungoides?

- Cutaneous T cell lymphoma | - Develops from Sezary's syndrome (once disease forms plaques, nodules, or lymphadenopathy it is called MF)

What is Sezary's syndrome?

- Cutaneous T cell lymphoma - Most present w/ill defined diffuse red rash ("parapsoriasis") - Called MF once disease forms plaques, nodules, or lymphadenopathy

Treatment of Sezary's/MF

Similar to psoriasis - light therapy (UVB) with or w/o psoralens or topical corticosteroids

What type of lymphoma does NOT follow the rule that big cells in lymphomas are bad?

Anaplastic large cell lymphoma

Describe Alk positive Anaplastic Large Cell lymphoma

- Usually disease of 40 or younger | - Responds to CHOP with 75% 5 yr DFS

Describe Alk negative Anaplastic Large Cell lymphoma

- Disease of elderly - Skin only involvement - Usually indolent, but incurable - Poorly responsive to CHOP and poor prognosis

Which type of anaplastic large cell lymphoma has a worse prognosis?

Alk negative

Describe nasal NK T cell lymphoma

- Asians - A/w EBV - Usually presents w/ulcerative lesion of nasal septum or sinuses - Chemo and RT therapy

How does Hodgkin's lymphoma present?

- Central lymphadenopathy - Fever, wt loss, sweats - Spreads from one nodal group to adjacent groups

Epidemiology of Hodgkin's lymphoma

- 50% cases a/w EBV | - Bimodal frequency (peak at 30 yo and 70 yo)

Types of Hodgkin's lymphoma

Classical and nodular

Subtypes of classical Hodgkin's lymphoma

- Lymphocyte rich - Nodular sclerosis (MC) - Mix cellularity - Lymphocyte depleted

MC type of classical Hodgkin's lymphoma?

Nodular sclerosing

Describe nodular sclerosing Hodgkin's lymphoma

- MC subtype of classical HL | - Mediastinum involvement of young adults

Most aggressive variety of HL?

Lymphocyte depleted subtype of classical HL

Which type of HL is MC in HIV pts?

Lymphocyte depleted subtype of classical HL

Standard treatment for HL?

ABVD given every 2 weeks IV for 8-12 cycles | Adriamycin, Bleomycin, Vinblastine, DTIC

Prognosis of HL?

Very curable

Which malignancy has a high cure rate so that survivorship is a huge issue to consider?

Hodgkin's lymphoma

Myeloma and Waldenstrom's are diseases of what?

Mature functioning B cells

Myeloma and Waldenstrom's are characterized by what?

Overproduction of immunoglobulins (from mature functioning B cells)

Which IG is overproduced in Waldenstrom's?

IgM

Which IG is overproduced in myeloma?

IgG | can be IgA or rarely IgD

Epidemiology of myeloma

- Median age at onset is 65 yo | - 2x MC in AAs

Prognosis of myeloma

Incurable but remission duration is improving

Diagnosis of myeloma

- CRAB (hypercalcemia, renal failure, anemia, bone lesions) - 10% or higher plasma cells in BM - 3 g/dl or more serum IgG/IgA

Define MGUS

Monoclonal gammopathy of unclear significance (some progress to myeloma, most never do)

Define smoldering myeloma

Disease between true myeloma and MGUS (50% develop myeloma within 5 yrs)

Treatment of MGUS or smoldering myeloma

No standard tx - close observation

Treatment of myeloma

- Minimize end organ damage - Consider HSCT (if yes, avoid alkylating agents) - If not, alkylating agent plus biological response modifier

Describe Waldenstrom's

- Usually 65 yo or older - IgM secretion by malignant cells - CD 19 and 20 positive

Clinical presentation of Waldenstrom's

Main symptoms are d/t the hyperviscosity caused by elevated IgM - lethargy, confusion, anemia, peripheral neuropathy

Treatment of Waldenstrom's

- Plasmapharesis to control levels of IgM until chemo can be effective - Rituximab plus Cyclophosphamide