Chapter 14 - Lymphoid Tissue Disorders Flashcards
Where in the lymphoid tissues are T cells located?
T cells: paracortex, thymus
Where in the lymphoid tissues are B cells located?
B cells: germinal follicles
Where in the lymphoid tissues are histiocytes located?
Histiocytes: sinuses, skin (Langerhans cell)
What age ranges are affected by benign or malignant nodal enlargement?
Nodal enlargement: 30 usually malignant
What is the most common cause of nodal malignancy?
Metastasis MC nodal malignancy
What does painful lymphadenopathy imply?
Painful lymphadenopathy: inflammation
What are the most common sites of localized painful lymphadenopathy?
Anterior cervical nodes/inguinal nodes MC site localized painful adenopathy
Name two causes of generalized painful lymphadenopathy.
Generalized painful adenopathy: SLE, infectious mono
What are the causes of painless lymphadenopathy?
Painless lymphadenopathy: metastasis or primary malignant lymphoma
What is a possible cause of painless axillary nodes in a woman?
Painless axillary nodes woman: metastatic breast cancer
What are the malignant causes of generalized painless lymphadenopathy?
Generalized painless adenopathy: acute/chronic leukemia; follicular B-cell lymphoma
What does left supraclavicular node metastasis indicate?
Left supraclavicular node metastasis: stomach/pancreatic carcinoma
What does hilar node metastasis indicate?
Hilar node metastasis: lung cancer
What is a cause of pre-aortic node metastasis in men?
Pre-aortic node metastasis: testicular cancer
What are causes of inguinal node metastasis?
Inguinal node metastasis men/women: penis/vulvar squamous cancer
Describe follicular hyperplasia.
Follicular hyperplasia: prominent germinal follicles
What is a key finding in dermatopathic lymphadenitis?
Dermatopathic lymphadenitis: melanin pigment in macrophages
What are the characteristics of cat scratch disease?
Cat-scratch disease: B. henselae; granulomatous microabscesses
What is toxoplasmosis?
Toxoplasmosis: mononucleosis-like syndrome with painful cervical adenopathy
Describe the characteristics of tularemia.
Tularemia: zoonosis (rabbits); F. tularensis; ulceroglandular MC type
What causes the plague?
Plague: Y. pestis; Yop gene protein products inhibit phagocytosis and kill phagocytes
Describe the transmission of the plague.
Transmission: bite of infected fleas that have bitten infected rodents (reservoir of bacterium)
Where does the bubonic plague mainly occur?
Bubonic plague mainly in Western U.S.
What are the three main presentations of the plague?
Bubonic plaque (MC), septicemic plague, pneumonic plague
If a flea transmitting Y. pestis bites a human leg, which nodes will be involved?
Bubonic plague: bite on leg; inguinal node involvement
What occurs during inguinal enlargement in the plague?
Inguinal node enlargement: bacteria proliferate, cause edema but few inflammatory cells present; nodes rupture
What are the characteristics of the septicemic plague?
Septicemic plague: endotoxemia; massive organ involvement; septicemia
What is a good prognostic sign in breast cancer?
Sinus histiocytosis in axillary nodes—good prognostic sign in breast cancer
Describe the origin of the majority of non-Hodgkin lymphomas.
NHL: majority B-cell origin
What are the extranodal sites in NHL?
Extranodal sites: stomach (MC), CNS, Peyer patches
What is the most common malignant lymphoma in adults and children?
NHL: MC malignant lymphoma adults/children
Which viruses are risk factors for NHL?
EBV: Burkitt lymphoma, primary CNS lymphoma (HIV)
HTLV-1: adult T-cell leukemia/lymphoma
HCV: B cell lymphoma
H. pylori is a risk factor for which type of lymphoma?
H. pylori: low-grade malignant lymphoma in stomach
Which autoimmune diseases are risk factors for NHL?
NHL autoimmune disease: Sjögren syndrome, Hashimoto thyroiditis
Describe the pathogenesis of NHL.
NHL: mutation blocks B/T cells at specific stage of development
Precursor T-cell lymphoblastic lymphoma/leukemia is common in which age group?
Precursor T-cell lymphoblastic lymphoma/leukemia: common in children
Describe the characteristics of mycosis fungoides.
MF: CD4 TH cell neoplasm; skin involvement (Pautrier microabscesses)
What is Sézary syndrome?
Sézary syndrome: MF in leukemic phase
Which gender is more affected by nodular sclerosing Hodgkin lymphoma?
Nodular sclerosing HL: female dominant
Describe the bimodal age distribution of HL.
Bimodal age distribution: 15–34 years old and >50 years old
EBV is associated with which type of HL?
EBV: association with mixed cellularity/lymphocyte depletion/lymphocyte rich HL
What kind of immunity is affected in HL?
HL: defects in CMI; anergy
What is the most common type of HL?
Nodular sclerosing classical HL: MC type of HL
What is important in HL pathogenesis?
Activated NF-κB important in HL pathogenesis
What is the neoplastic cell of HL?
RS cell neoplastic cell of HL
What is required to diagnose HL?
RS cell required to diagnose HL
What are the clinical findings in HL?
HL: fever, weight loss, night sweats; pruritus
What is important in determining the prognosis in HL?
Prognosis: stage more important than type of HL
What are the clinical findings in nodular sclerosis HL?
Nodular sclerosis HL: anterior mediastinal mass + single group of nodes above diaphragm
What are the risks of treatment in HL?
Rx HL: ↑risk 2nd malignancies (AML, NHL)
What are the characteristics of histiocytes?
Histiocytes: CD1+; Birbeck granules
What are the characteristics of Letterer-Siwe disease?
Letterer-Siwe: malignant; skin involvement; lytic bone lesions
What is the classic triad of Hand-Schüller-Christian disease?
HSC: lytic skull lesions, CDI, exophthalmos
What are the chracteristics of eosinophilic granuloma?
Eosinophilic granuloma: benign histiocytosis; unifocal lytic lesions in bone
What are the signs and symptoms of mast cell disorders?
Mast cell disease: pruritus, swelling, hyperpigmentation
What are the characteristics of urticaria pigmentosum?
UP: dermatographism; lesions remain hyperpigmented
What type of granules do mast cells have?
Mast cells: metachromatic granules
What is increased in monoclonal gammopathies?
MG: single M protein + light chain
What type of M protein is present in the majority of MGs?
Majority have IgG M protein
What are Bence Jones proteins?
BJ protein: light chains in urine
What does the serum protein electrophoresis show in MGs?
SPE shows monoclonal spike
Testing the serum for free light chains is more sensitive than which type of tests?
Serum free light chains: more sensitive than urine tests
Which age groups and races are affected by multiple myeloma?
MM: rare whites
List the top three most common types of multiple myeloma in descending order.
MM: IgG > IgA > light chain myeloma
What does evidence of end-organ damage include in multiple myeloma?
CRAB: calcium elevation, renal insufficiency, anemia, and bone lesions
Describe the evolution of normal plasma cells to MM.
MM: MM: normal plasma cell → MGUS → myeloma
What are the pathologic findings and skeletal system findings in MM?
MM: lytic lesions, plasma cells >10%; pathologic fractures, hypercalcemia
What is the most common site of bone involvement in MM?
MM: vertebrae MC bone site
Describe the renal disease caused by Bence Jones proteins in MM.
BJ renal disease in MM: proteinaceous casts with multinucleated giant cell reaction
MM is associated with which type of amyloidosis?
MM: association with primary (AL) amyloidosis
What are the hematologic findings in MM?
MM: anemia with rouleaux, ↑bleeding time (platelet aggregation defect)
What are the common causes of death in MM?
MM: sepsis/renal failure common causes of death
What is the most common MG?
MGUS: MC monoclonal gammopathy
What is present in the red and white pulp of the spleen?
Red pulp: fixed macrophages
White pulp: B and T cells
What are the functions of the spleen?
Functions: blood filtration (encapsulated bacteria), antigen trapping/processing, platelet reservoir, EMH
What are the characteristics of Gaucher disease?
Gaucher disease: ↓glucocerebrosidase, ↑glucocerebroside, macrophages fibrillary appearance
What are the characteristics of Niemann-Pick disease?
Niemann-Pick: ↓sphingomyelinase, ↑sphingomyelin, macrophages soap bubble appearance
What are the clinical findings in massive splenomegaly?
Massive splenomegaly: infarctions with pain, friction rub, and left-sided pleural effusion
What is the gross finding in splenomegaly in cirrhosis with portal hypertension?
Splenomegaly in cirrhosis with portal hypertension: sugar-coated spleen
What is hypersplenism?
Hypersplenism: destruction of hematopoietic cells produces cytopenias
What is the most common cause of hypersplenism?
PH MCC hypersplenism
Splenic dysfunction increases the risk for what?
Splenic dysfunction: ↑risk for S. pneumoniae sepsis; HJ bodies
Describe the mechanisms by which encapsulated pathogens cause infection in splenic dysfunction.
Mechanisms: ↓IgM, ↓tuftsin, ↓splenic macrophages