IS: Lymphoma and Polymyositis

Term
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NORMAL MUSCLE

-________ fiber size

-no significant ________

-________ internal nuclei

-_________ fiber distribution (ATPase, NADH)
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Terms in this set (23)
NORMAL MUSCLE VS. ABNORMAL (MYOPATHIC) CHANGES


Variation in fiber size (use cross-sections)
-Normal muscle should have little variation in fiber size. This is best seen on cross sections.

-Abnormal muscle can have much variation in size. Pattern or distribution of the changes can be helpful in identifying the process.

Number of internal nuclei
-In normal muscle internal nuclei should be seen in only a few fibers (i.e.. <4%)

-In many myopathic conditions, internal nuclei can be seen in _______.

Amount of inflammation and necrosis
-In normal muscle, there should be no inflammation or necrosis.

-In inflammatory myopathies, both inflammation and necrosis of muscle fibers can be prominent.
MYOPATHIC PROCESSES

Processes are directed at and/or involve skeletal muscle directly.

See above for myopathic changes.

_________>Distal weakness

Categories of myopathic disease (per Robbins, Chapter 27)
-Inflammatory (Autoimmune)
-Toxic
-Genetic
--Dystrophic (e.g. Becker and Duchenne dystrophinopathy)
--Metabolic (including mitochondrial and enzyme deficiencies (e.g. phosphorylase)
--Congenital (hypotonia/"floppy baby")
Steroids are the primary therapies for both dermatomyositis and polymyositis. This is directed at the inflammation. However, inclusion body myositis (another inflammatory myopathy) does not respond to corticosteroids.

Note that steroids can case muscle problems. In particular, steroid therapy can result in Type 2 fiber atrophy. Thus, steroids should be avoided in inclusion body myositis.
LOOK AT CONCEPT MAPS ON SLIDES 23-24 REVIEW**----------SOME BASIC DIFFERENCES BETWEEN LEUKEMIA AND LYMPHOMA Leukemia -Malignancy of ________ cells -Starts in _______, can spread to blood, lymph nodes -Acute or chronic types Lymphoma -Malignancy of _______ cells -Starts in __________, can spread to blood, marrow -Hodgkin or_______ typesmyeloid or lymphoid bone marrow lymphoid lymph nodes non-Hodgkin: focus for this DSACD (CLUSTER OF DIFFERENTIATION) MOLECULES CD (cluster of differentiation) molecules are cell surface markers useful for the identification and characterization of leukocytes (white blood cells). The CD nomenclature was developed and is maintained through the HLDA (Human Leukocyte Differentiation Antigens) workshop started in 1982. Some notable CD markers: -CD3 ___________ -CD5 ___________ -CD10 ___________ -CD20 ___________ -CD23 ___________T cell marker T cell marker, presence of CD5 on B cells is aberrant Immature pre B and pre T cells, follicular center B cells B cell marker Involved in B cell activation and growthNON-HODGKIN LYMPHOMA Malignant proliferation of ________ in lymph nodes Can see __________ -Note: As opposed to Hodgkin lymphoma which generally shows only contiguous spread Many subtypes Most are __________lymphoid cells non-contiguous spread or contiguous spread B cell originNON-HODGKIN LYMPHOMA SYMPTOMS _________, firm lymphadenopathy May have extranodal involvement May have "B" symptoms: __________Painless fever, night sweats, weight loss (>10%)NON-HODGKIN LYMPHOMA Low-grade: Adults, Slow progression Small, mature and monotonous cells Typically not curable Small lymphocytic lymphoma + Follicular High-grade: Adults and ________ More rapid progression Larger and more varied cells May be curable; highly dividing cells so radiation hits it more Diffuse large cell B lymphoma + mantle cell lymphomachildrenFollicular lymphoma IgH; can see the number 14 18 letters in follicular lymphoma t(14; 18); typical chromosome translocation----------Mantle cell lymphoma t(11; 14) Does not have CD23-----------CASE 1 A 69-year-old man visits his physician because he has experienced fatigue and noticed lumps in his right axilla and along the right side of his neck for the past 6 months. He has right cerival and axillary nontender lymphadenopathy. One of the lymph nodes is biopsied and he is found to have a neoplasm composed of B cells that are CD5 positive and CD23 positive. What is the most likely diagnosis?Small lymphocytic lymphoma CD5+, CD20+ and CD23+ B cells, but not CD10- Most have lymphadenopathy and bone marrow involvement but not B symptomsCase 2 A 62-year-old woman visits her physician because she has experienced fatigue, fever, night sweats, and noticed lumps in her neck for the past 3 months. She has bilateral cervical nontender lymphadenopathy. One of the lymph nodes is biopsied and she is found to have a neoplasm composed of B cells that are CD10 positive and have a t(14:18) translocation. What is the most likely diagnosis?Follicular lymphoma T(14; 18) translocation Older people generally CD10+ and CD20+ B cells, but also CD5-, CD23 variable Form crowded back-to-back folliclesCase 3 A 70-year-old man visits his physician due to rapidly enlarging painless lumps in the inguinal and axillary regions bilaterally. He denies fever, night sweats, or weight loss. He has bilateral inguinal and axillary nontender lymphadenopathy with enlargement of his spleen and liver. One of the lymph nodes is biopsied and he is found to have a neoplasm composed of large B cells that are CD5 negative, CD 10 negative, CD23 negative, and CD20 positive. What is the most likely diagnosis?Diffuse large B cell lymphoma CD20+ B cells Lower median survival rate, but cured at higher rate Usually 5x the size of normal lymphocytes and are pleomorphicCase 4 A 64-year-old man visits his physician due to painless lumps in the axillae bilaterally. He denies fever, night sweats, or weight loss. He has bilateral axillary and supraclavicular nontender lymphadenopathy. One of the lymph nodes is biopsied and he is found to have a neoplasm composed of small B cells that are CD5 positive, CD23 negative, and have a t(11:14) translocation What is the most likely diagnosis?Mantle cell lymphoma CD5+ and CD20+ Differs from SLL, which is generally positive for CD23 Characteristic translocation b/w chromosome 11 and 14 Small to medium sized lymphocytesWhich is more likely to be curable?High grade and fast growing lymphoma