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