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79 Cards in this Set
- Front
- Back
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What is Hodgkin's Lymphoma?
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A cancer involving the spleen and lymphatic system. (1932 - Thomas Hodgkin's first described)
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What binucleate or multinucleated giant cells were described in 1898 & 1902; there presence now is critical in a Hodgkin's diagnosis
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Reed-Sternberg cell - has bilobed nucleus w/ 2 large, prominent nucleoli that give an Owl's eye appearance.
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What percentage of these malignant Reed-Sternberg cells are accounted for in the tumor's total cell population?
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0.1 - 10% - this is what makes Hodgkin's Lymphoma unique
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What is a Hodgkin's tumor mostly composed of?
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Most of tumor is inflammatory cells & fibrosis - d/t cytokine release
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The univerally accepted WHO histologic classification system for HL is based largely from what other classification system?
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Rye Classification - divides HL into 2 broad classes
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What are the 2 broad classes of HL in the Rye classification system?
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1. Classical Hodgkin's Lymphoma (cHL)
2. Nodular Lymphocyte predominant Hodgkin's lymphoma (NLPHL) |
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What type of cells does NLPHL consist of?
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Large multilobed nuclei termed
"popcorn cells". |
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T or F: cHL cells are hallmarked by the Reed-Sternberg cells
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True
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What 3 characteristics differentiate the 4 subtypes of cHL?
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1. tissue structure
2. fibrosis 3. inflammatory process |
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What are the 4 subtypes of cHL?
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1. Nodular Sclerosing HL (NSHL)
2. Mixed Cellularity HL (MCHL) 3. Lymphocyte-Rich Classical HL (LRCHL) 4. Lymphocyte-Depleted HL (LDHL) |
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NLPHL class most closely resembles what subtype of cHL in regards Histology?
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Lymphocyte-Rich Classical HL (LRCHL).
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What demographic is most commonly affected with NLPHL?
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10 years and younger; males
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How does NLPHL typically present in terms of the extent of disease?
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Localized and asymptomatic
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What subtype of cHL is the most common?
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Nodular Sclerosis HL;
- 45% of all cases in kids up to 8yrs of age; - 80% of all cases in older kids & teens |
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What lymph nodes are commonly involved in NSHL?
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Lower cervical lymph nodes
Supraclavicular lymph nodes Mediastinal lymph nodes |
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What is the radiographic appearance of NSHL lymph nodes?
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1. Lymph node capsule appears thickened
2. Lymphoid tissue appears nodular - Radiographic appearance looks normal after treatment |
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Which subtype of cHL is most commonly in kids under 10yrs & accounts for 30% of all cases?
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Mixed Cellularity Hodgkin's Lymphoma
(MCHL) |
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T or F: MCHL is frequently advanced upon diagnosis
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True - usu. involved extranodal regions
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What histologic characteristics are usually seen in MCHL?
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Lymph node is diffusely effaced & contains a number of Reed-Sternberg cells.
Inflammatory background of lymphocytes, plasma cells, eosinophils, histiocytes, & malignant reticular cells. |
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What subtype of cHL affects approx. 5% of pt's w/HL?
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Lymphocyte-Rich Classical Hodgkin's Lymphoma (LRCHL)
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How does LRCHL typically present in terms of histology and disease?
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Localized disease w/Reed-Sternberg cells in a background of mostly small B-lymphocytes
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T or F: Lymphocyte-depleted HL is associated with SCIDs patients
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False - associate with HIV patients
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LDHL (Lymphocyte-Depleted HL) is common or rare subtype of cHL?
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Rare
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What disease status is commonly seen in LDHL?
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Late-stage, extensive disease
Widespread in bones & BM |
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How is LDHL characterized histologically?
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- many Reed-Sternberg cells
- few lymphocytes - diffuse fibrosis & necrosis |
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What percentage of childhood cancers does HL account for?
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6%
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What is the male to female ratio for HL diagnosis?
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Male to female dominance of 4:1 in children less than 8years
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T or F: The male to female dominance in incidence increases by age 10yrs
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False: the ratio decreases to be more equal, approx. 1.3:1 male to female ratio
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How does HL age distribution uniquely differ than other types of childhood cancer?
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it is based on
- geography - ethnicity - socioeconomic status |
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What is the peak age of incidence in industrialized countries? Developing countries?
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Industrialized countries - early age peak occurs mid to late 20s, second peak after age 50.
Developing countries - early peak occurs before adolescence |
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What 3 forms of HL have distinctly emerged?
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1. Childhood = 14yrs & younger
2. Young adult = 15-34yrs 3. Older adult = 55-74yrs |
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T or F: There is an increased incidence of the childhood form of HL in children of a higher socioeconomic status
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False - incr'd incidence of childhood form in lower socioeconomic status
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What form of HL is there an increased incidence in the higher socioeconomic status group?
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Young adult form
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Can Hodgkin's lymphoma be genetic?
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Yes - but it is rare
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Children with what disorders also have an increased incidence of HL?
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Children w/immunologic disorders
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What virus has been associate with Hodgkin's Lymphoma?
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EBV - found in some Reed-Sternberg cells (15-20% of teens/young adults of EBV positive HL)
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What is the typical clinical presentation of HL?
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80% = painless adenopathy; usu. supraclavicular & cervical nodes
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What do the lymph nodes characteristically feel like on exam?
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Firm, rubbery texture, sensitive to palpation
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What do 2/3 of HL patients present with?
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Mediastinal involvement; symptoms possibly d/t pressure on trachea & bronchi
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What percentage of U.S. children & teens present with only involvement of lymph nodes or spleen or both?
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80- 85% = Stages I thru III.
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What percentage of HL patients will present with Stage IV disease?
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15-20%
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What organs does Stage IV HL affect?
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Non-continuous extranodal involvement to lungs, bones, liver, and/or bone marrow
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25% of patients may have systemic symptoms of HL that consist of what symptoms?
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Fatigue, fever, weight loss, and night sweats
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T or F: Constitutional symptoms of HL correlate with prognosis
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True = they are referred to as B symptoms
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What are the B symptoms that correlate with pronosis?
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1. Unexplained fever >38C for >3days
2. Unexplained wt. loss of 10% in 6months of diagnosis 3. Drenching night sweats |
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T or F: When B symptoms are present, it places them in a different disease category when staging
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True = B symptoms place patient in B category when staging
(if B symptoms are absent, pt's are placed in "A" category when staging) |
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If a patient presents with mild to severe pruritus, what is their likely stage of disease?
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Advance-staged disease pt's more commonly reported pruritus.
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In Stage I Hodgkin's lymphoma how many lymph node regions are involved?
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One region or one region w/extension from node to adjacent extralymphatic region (IE).
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T or F: Stage II HL involves 2 lymph node regions on opposite sides of the diaphragm
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False - Stage II is on same side of diaphragm either with or without extension into extralymphatic adjacent organs (IIE)
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In Stage III Hodgkin's lymphoma lymph node regions are involved on both sides of the diaphragm and what potential extralymphatic organ involvement?
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If extension is involved it can be to extralymphatic organ (IIIE), involvement of spleen (IIIS) or both (IIE+S)
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Does stage IV HL always involve lymph nodes?
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No - but one or more noncontiguous involvement of extralymphatic organs or tissue (w/ or w/out lymph nodes)
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What is crucial for diagnostic evaluation of a potential HL patient?
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Examination of lymph node chains size and measurements.
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What chemistry labs may be elevated in the blood of a HL patient at diagnosis?
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Copper and Ferritin
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What defines bulky disease in HL?
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Lymphadenopathy greater than or equal to 33% of the max intrathoracic cavity
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What stage HL patients do NOT need a BM biopsy upon diagnosis?
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IA/IIA
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Is surgical staging of lymph node sampling a common diagnostic tool in HL?
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No
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What is the approximate cure rate of children with HL?
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90-95%
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What are favorable prognostic indicators upon a HL diagnosis?
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1. Localized nodal involvement
2. No B symptoms 3. No bulky disease |
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What are unfavorable prognostic indicators in HL?
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1. Advance-stage disease
2. Presence of B symptoms 3. Bulky disease 4. Extra-nodal involvement 5. Male gender 6. Elevated Sed rate |
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Is HL ever treated with radiation alone?
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Rarely - typically chemo combination; occasionally chemo alone is warranted
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How often is a chemotherapy cycle repeated in HL treatment regimens?
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Every 21-28 days, then re-evaluated disease after several cycles
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Commonly used chemo regimen HL:
What chemo's are used in VAMP? |
Vincristine
Adriamycin MTX Prednisone |
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Commonly used chemo regimen HL:
What chemo is used in COPP? |
Cyclophosphamide
Vincristine Procarbazine Prednisone |
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Commonly used chemo regimen HL:
What chemo is used in ABVD? |
Adriamycin
Bleomycin Vinblastine Dacarbazine |
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Commonly used chemo regimen HL:
What gender is OPPA given to, and what chemo is used in OPPA? |
Females
Vincristine Prednisone Procarbazine Adriamycin |
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Commonly used chemo regimen HL:
What gender is OEPA given to, and what chemo is used in OEPA? |
Males
Vincristine Etoposide Prednisone Adriamycin |
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Commonly used chemo regimen HL:
What chemo is used in COPP/ABV? |
Cyclophosphamide
Vincristine Procarbazine Prednisone Adriamycin Bleomycin Vinblastine |
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Commonly used chemo regimen HL:
What chemo is used in ABVE-PC? |
Adriamycin
Bleomycin Vincristine Etoposide Prednisone Cyclophosphamide |
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Commonly used chemo regimen HL advance-stage:
What chemo is used in BEACOPP? |
Bleomycin
Etoposide Adriamycin Cyclophosphamide Vincristine Prednisone Procarbazine |
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What does LD-IFRT stand for regarding HL?
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Low dose - involved field radiation therapy
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What is LD-IFRT used for in HL?
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To achieve local control and minimize damage to non-cancerous tissue
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In what time frame to most relapses of HL typically occur?
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Within 3 years of diagnosis
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What is the overall long-term survival for patient who relapse with HL?
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ranges from 30-90%, depends on several factors
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What does salvage chemotherapy typically consist of in regards to HL?
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Chemo's not commonly used in initial treatment (ex., Cytarabine, carboplatin, cisplatin, Ifos, VP16, Vinorelbine, Gemcitibine, Vinblastine)
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What patients are considered high risk upon relapse?
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Those with
- refractory disease - relapse w/in 1yr of completing therapy - multiple relapses |
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What is typically considered treatmetn of choice for relapse or recurrent HL patients?
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Myeloablative chemo w/ AUTO SCT
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What is the progression free survival rate of relapsed/recurrent disease patient's who receive SCT?
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Approx. 30-65% progression free survival
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What are the 4 goals of Nrsg Assessment and Interventions in regards to HL?
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Pt will experience minimal complication r/t 1. Onset of the tumor; 2. Treatment.
Pt. & family will 3. be adequately informed re: diagnosis, disease, & treatment; 4. cope adequately with diagnosis |
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How is tumor lysis syndrome managed?
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IV hydration w/alkalinization
Allopurinol Monitor I&Os |
