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35 Cards in this Set

  • Front
  • Back
what is the overall incidence of non-hodgkin lymphoma?
20/100'000
what is the median age for NHL?
65 years
what is the M:F ratio in NHL?
M:F = 1.5:1
what are principal risk factors for development of a NHL?
congenital or acquired immunodeficiencies

late complications after radiation

oncogenic viruses
what are the oncogenic viruses associated with NHL?
HTLV-1 in adult T-cell lymphoma/leukemia (ATLL)

EBV in endemic and HIV-associated Burkitt lymphoma, less commonly in the sporadic variant of Burkitt lymphoma

HHV-8 in Kaposi's sarcoma and primary effusion lymphoma (body-cavity-based lymphoma BCBL)
what are other infections associated wtih NHL?
Helicobacter pylori -> gastric MALT lymphoma

Chlamydia psitacci --> ocular adnexal lymphomas

Hepatitis C virus
what is the hallmark of the pathogenesis in NHL?
chromosomal translocations are the genetic hallmark of lymphoid malignancies
in what pathogenetic mechanisms are NHL different from those in solid tumors?
genome of the malignant cell is relatively stable in contrast to the generalized random instability especially of solid tumors of epithelial origin

generally there is a lack of microsatellite instability, the hallmark in some hereditary sydromes (leading to predisposition to cancer) and in most sporadic tumors

the genome of lymphoma cells is characterized by few non-random chromosomal abnormalities, commonly balanced chromosomal translocations
how are NHL staged?
adapted Ann Arbor classification

Stage I single lymph node region Stage IE single extralymphatic organ or site

Stage II more than one lymph node region on the same side of the diaphragm
Stage IIE localized involvement of an extralymphatic organ or site

Stage III lymph node involvement of both sides of the diaphragm
Stage IIIE localized involvement of an extralymphatic organ or site
Stage IIIS involvement of spleen
Stage IIIES both

Stage IV disseminated involvement of extralymphatic organ with or without lymph node involvement

additional notes are A for asymptomatic or B for presence of B symptoms
what are prognostic tools in NHL?
International Prognostic Index and variants, originally for high-grade lymphomas

criteria are age>60, abnormal LDH, limited performance status, Ann Arbor stage III/IV and number of extranodal disease sites >1

exceptions are CLL and plasmocytoma
what is the median age at diagnosis in NHL?
sixth decade, exceptions are Burkitt lymphoma [and acute lympoblastic lymphoma (ALL)]
what proportion of lymphomas are NHL?
85%
what proportion of NHL are of B-cell origin
85%
which NHL are classified as indolent?
indolent = low-grade

follicular lymphoma
marginal zone lymphoma (MALT, splenic, extranodal)
lymphoplasmacytic lymphoma - immunocytoma - Waldenström makroglobulinemia
what proportion of NHL, irrespective of cell origin are primarily extranodal?
20%
what proportion of peripheral T-NHL are primarily extranodal?
80%
which is the organ most commonly affected by T-NHL
the skin
which are the most frequent extranodal sites affected in NHL?
stomach, intestine, skin and brain
which autoimmune disorders are commonly accompanied by an increased risk for NHL?
Sjoegren's syndrome
Hashimoto thyroiditis
Rheumatoid arthritis
Celiac disease
is exposure to ionizing radiation a risk factor for NHL?
no, most commonly identified malignancy following exposure to ionizing radiation is leukemia
what are groups of differential diagnosis in NHL?
drug-induced pseudolymphoma syndrome

mycobacterial infections, especially in immunocompromised

fungal infections (eg histoplasmosis, cryptococcosis)
what are salvage chemotherapy regimens commonly used in recurrent aggressive NHL?
DHAP
Dexamethasone, high-dose cytarabine, cisplatin

ESHAP
etoposide, methylprednisolone, high-dose cytarabine, cisplatin

EPOCH
etoposide, vincristine, doxorubicin, cyclophosphamide, prednisone
which epidemiologic phenomena can be seen with advancing age in NHL?
NHL incidence rising with advancing age, additionally transition from high-grade to low-grade NHL with rising age
what are the NHL commonly classified as low-grade?
Follicular Lymphoma

Marginal Zone Lymphoma

Small Lymphocytic Lymphoma (cf CLL)
what are the NHL commonly classified as intermediate-grade?
DLBCL

Follicular Lymphoma grade III

Mantle Cell Lymphoma
what are the NHL commonly classified as high-grade?
Burkitt and Burkitt-like Lymphoma (BL)

Lymphoblastic Lymphoma (LL) = T-cell variant of ALL
what are the three most frequently involved sites of extranodal NHL?
gastrointestinal tract, skin and brain
which cytostatic drugs penetrate the blood barrier?
methotrexate, cytarabine, nitrosourea alkylating agents
what are mature clonal B-cell neoplasms with accumulation of neoplastic plasmacytoid cells with excretion of a monoclonal Ig molecule?
MM, extramedullary and solitary plasmocytoma, MGUS, Waldenström macroglobulinemia
what are other hematologic malignancies in which monoclonal gammopathies can occur?
B-CLL
indolent NHL
cold agglutinin disease
amyloidosis
what are differences between HL and NHL concerning node involvement?
node involvement is typically localized to a single axial group of nodes in HL, while involvement of multiple peripheral nodes is more frequent in NHL
what are differences between HL and NHL concerning dissemination?
spread in HL is typically orderly by contiguity while NHL show a noncontiguous spread
what are differences between HL and NHL concerning mesenteric node and Waldeyer ring involvement?
mesenteric nodes and Waldeyer ring are rarely involved in HL, while they are commonly involved in NHL
what are differences between HL and NHL concerning extranodal presentation?
extranodal presentation is rare in HL, while in NHL it is common
what are the most common fungal infections in leukemic patients?
aspergillus, candida, zygomycetes (mucorales), fusarium