Myeloma and Lymphomas Flashcards
What is the origin of most lymphomas?
B cell
What do B cells produce?
Immunoglobulin antibodies
What do T cells produce?
Cytokines
Which types of NHL are typically more aggressive but sometimes curable?
Larger cells
Which types of NHL are typically less aggressive but generally incurable?
Smaller cells
How does lymphadenopathy progress in NHL?
Spreads non-contiguously from peripheral LN groups inward toward central LN groups
How does lymphadenopathy progress in Hodgkin’s lymphoma?
Spreads contiguously from central LN groups outward
Describe the diagnostic lymph node in NHL
Most of the cells are malignant
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
What are Reed-Sternberg cells?
- Malignant cells in a lymph node of Hodgkin’s lymphoma
- “Owl eyed” appearance
- CD 30 positive
MC adult leukemia?
CLL/SLL
Describe CLL/SLL
Identical - differ only in how they are initially diagnosed (SLL if found in a lymph node)
Typical lab findings of CLL
- Absolute lymphocyte count over 5000 consisting of small mature B cells
- CD 19 and 20 positive
- CD 5 positive
Treatment of CLL/SLL
- Not curable with chemo
- May be cured with allogeneic SCT (rarely used d/t morbidity)
What are CLL-like lymphoid leukemias?
- Hairy cell leukemia
- Splenic marginal zone lymphoma/leukemia
Classic lab finding of hairy cell leukemia
- Small lymphocytes w/hairy projections
- CD 19, 20, 11c, 103 positive
Treatment of hairy cell leukemia
Purine analogs (80% CR, 60% long term CR)
Gastric MALTs are frequently caused by what?
H pylori infections
Describe MALT lymphomas
Low grade lymphomas usually of lymphoid tissue a/w exocrine glandular tissue
What can cure some early stage MALT lymphomas?
Treatment of H pylori infections
What are follicular lymphomas characterized by?
Lymphocytes and CD 10, 20
MC adult lymphoma?
DLBCL
Describe DLBCL
- MC adult lymphoma
- Aggressive, frequently involves extra-nodal sites
- CD 19 and 20 positive
Standard treatment of DLBCL?
R-CHOP
Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone
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