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Hematology
Lymphoma
44
Medical
Graduate
11/24/2009

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Term
What is a congenital B lymphocyte deficiency?
Definition
Bruton's (x-linked) agammaglobulinemia - dec. or absent B lymphocytes and dec. ability to prod. Ab
Term
What are pts w/ Bruton's agammaglobulinemia particularly susceptible to? Who is affected by this d/o?
Definition
Infxns of throat, skin, middle ear, and lungs. Males.
Term
What is a T lymphocyte def. that begins in fetal dev., but is not genetic?
Definition
DiGeorge syndrome
Term
Why do pts w/ DiGeorge syndrome have T lymphocyte def.? What types of infxns are they susceptible to?
Definition
Absent or underdev. thymus. Viruses, fungi, and some bacteria.
Term
What physical appearance is characteristic of DiGeorge syndrome?
Definition
Low set ears, small receding jawbone, and widely spaced eyes.
Term
what are the most common causes of aquired immunodeficiency?
Definition
Malnutrition, some CA, chicken pox, CMV, Rubella, Measles, TB, Mono, chronic hepatitis, lupus, bact. and fungal infxns.
Term
Name 3 types of lymphocytes.
Definition
1) T cells (80-90%) 2) B cells 3) NK cells (don't req. prior antigenic exposure)
Term
what is the promary function of a lymph node?
Definition
Filtration
Term
What is the most common cause of lymphoma in young adults?
Definition
Hodgkin's lymphoma (HL)
Term
Peak age of incidence of HL?
Definition
15-35yo and >50yo
Term
Is HL more common in men or women? What race is it less common?
Definition
Men, African American
Term
Prognosis of HL?
Definition
>75% new dx can be cured w/ chemo/XRT
Term
What virus is frequently present in the malignant cells of HL?
Definition
EBV
Term
Other than EBV, what viruses are belived to be linked to the dev. of HL?
Definition
Chicken pox, Rubella, and Pertussis
Term
The presence of what on biopsy are key findings that lead to the dx of HL vs. NHL?
Definition
Reed-Stenberg (RS) cells.
Term
Desccribe RS cells.
Definition
Orig. from B cells, unique b/c only a minority of cell population, lg. size and binucleated
Term
Name the 4 pathologic variants of pathologic subtype of HL. which is the most common?
Definition
1) Nodular Sclerosis (most common) 2) Mixed cellularity 3) Lymphocyte rich 4) Lymphocyte depletion (rare)
Term
Describe nodular sclerosing type of HL.
Definition
Fibrous bands separate the node into nodules and lacunar RS cells
Term
Describe mixed cellularity type of HL.
Definition
RS cells easily identified and band forming sclerosis is absent
Term
Describe lymphocyte rich type of HL.
Definition
variants of RS cells w/ polylobated nuclei (popcorn cells), classic RS cells absent
Term
Which gender is the lymphocyte rich type more common in? What is the prognosis?
Definition
Males. Excellent, peripheral nodes sparing the mediastinum
Term
Describe lymphocyte depleted type of HL. How common is it? Who does it most often affect?
Definition
Sheets of RS cells lacking inflammatory cells. Rare (<1%). elderly.
Term
Describe how lymph nodes present in HL.
Definition
Painless enlargement, usu. in neck and mediastinum.
Term
Describe the A and B component of staging of HL.
Definition
A - sx absent. B - sx present.
Term
What are the most common B sx of HL? (5)
Definition
Fever, night sweats, wt loss, dyspnea, gen. pruritus
Term
What does "bulky dz" mean in regards to HL?
Definition
Occupies >10cm or >33% of chest diameter on CXR
Term
What B sx indicate the progression of HL? (3)
Definition
Malaise, cachexia, and infections
Term
Describe the Ann Arbor staging system w/ Cotswald modifications for lymphoma.
Definition
Describe lymph node involvement - Stage I - IV
Term
What are the stages of Ann Arbor staging system?
Definition
I)Single node or region. II)2nd node region on same side of diaphragm. III)Node region on both sides of diaphragm. IV)Disseminated (multifocal) inv. 1 or more organs
Term
What is the other staging system used for lymphoma that divides pts into 3 groups?
Definition
Early favorable (stage I or II w/o risk factors), Early unfavorable (stage I or II w/ 1 or more RF), and Advanced (Stage III or IV )
Term
What is the gold standard of tx for favorable early-stage HL?
Definition
XRT + Chemo w/ Adriamycin (doxyrubicin), Bleomycin, Vinblastine, and Dacarbazine (ABVD)
Term
Which is more common, HL or non-hodgkin's lymphoma (NHL)?
Definition
NHL - 6th most common CA in the US
Term
What is the peak age of incidence of NHL?
Definition
appears in all ages (rarely in 1st yr of life), incidence inc. w/ age
Term
What is the etiology of NHL?
Definition
Unknown - poss viral cause (T cell leukemia-lymphoma virus, EBV, HIV)
Term
What are risk factors for NHL? (4)
Definition
1) Immunodeficiency 2) H/ pylori 3) Chemical exposure 4) h/o tx for HL
Term
How long after organ transplant does HL occur? NHL?
Definition
HL - 4yrs and NHL - 1yr
Term
What are the 3 classifications of NHL?
Definition
Low-grade, intermediate-grade, and high-grade
Term
Describe lymphadenopathy in low-grade NHL.
Definition
Waxing and waning or slowly progressing, rubbery, mobile, no overlying skin infiltration, painless
Term
What are common extranodal sites affected by NHL? What type of pt's w/ NHL may have CNS involvement?
Definition
Liver, spleen, and bone marrow. CNS rare except in HIV pts.
Term
Describe lymphadenopathy in intermediate and high grade NHL.
Definition
Abrupt onset w/ rapidely enlarging lymph nodes, hard and fixed w/ overlying skin infiltration
Term
Name 4 conditions that may be caused by lymph node masses in NHL?
Definition
1) Lymphedema 2) Uretal obs. 3) Vascular obs. (SVC syndrome, thrombophlebitis) 4) Epidural cord compression
Term
Describe the tx for NHL if the pt has limited dz and only one lymph node is involved?
Definition
Localized XRT
Term
What is the tx for high-risk NHL?
Definition
Autologous stem cell transplantation, chemo and/or XRT
Term
Name 4 risk factors for poor prognosis in NHL.
Definition
1) >60yo 2) Elev. LDH 3) Stage III or IV 4) Poor performance status
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