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189 Cards in this Set
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Leukemia
|
Neoplastic expansion of hematopoietic cells in blood and bone marrow
Anemia, infection, bleeding |
|
Acute Leukemia
|
expansion of immature cells
|
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Chronic Leukemia
|
expansion of matture/differentiated cells
|
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Meylogenous Leukemia
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From Myeloid precursors
|
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Lymphocytes Leukemia
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From lymphoid precursors
|
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Leukemia Etiology
|
Radiation, chemicals, hereditary
|
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Chromosomal Abnormalities in Leukemias
1. t(9;22) 2. t(8;21) 3. t(15;17) 4. inv(16) 5. del(5)del(7) |
1. CML, ALL --bad
2. AML --good 3. AML --good 4. AML-M4 5. Bad prognosis |
|
Granulocytic Sarcoma
|
Accumulation of immature myeloid blasts in organs
Skin, lymph nodes, brain, kidneys Infection, bleeding, anemia |
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Meylodysplastic Syndrome
|
-"pre-leukemia"
defective hematopoiesis elderly dyspoiesis <20% blasts in BM progress to AML Due to idiopathic or radiation or chemo |
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Pancytopenia
|
Decrease in all 3 cell lineages
Causes: aplastic anemia, drugs (chemo), viral (HCV, HIV, CMV), leukemia, vit B12 deficiency, tumor in BM |
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Multiple Myeloma
|
neoplasm of plasma cells (SPEP, UPEP)
lytic lesions in bones, fractures anemia, increased globulin renal failure, amyloidosis |
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Polycythemia
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increased concentration of RBC mass, HGB, HCT
secondary to chronic smoking, COPD, high altitude |
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Polycythemia Vera
|
increased RBC mass
hyperviscosity, thrombosis dizziness, headache, weakness splenomegaly phlebotomy, anti-coagulant, chemo |
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Thrombocythemia
|
Increase in number of PLT (>150,000 - 400,000)
Iron deficiency anemia, chronic infection, inflammation |
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Lymphoma
|
neoplastic expansion of lymphoid cells in lymph nodes presenting as a discrete tumor mass
|
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Hodgkin's Histology
|
Reed-Sternberg cells (large cells with 2+ nuclei with prominent central nucleoli)
Lacunar cells Nodular Sclerosis Mixed Cellularity Lymphoid Predominant Lymphocyte Depleted |
|
Hodgkin's Features
|
15-34 and >55
Infectious mono, EBV Immunocompromised Family history Alcohol sensitivity Persistent, itching skin |
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Hodgkin's Clinical Presentation
|
Large, painless, lymph node
B symptoms: fever, weight loss, night sweats Hepatosplenomegaly |
|
Hodgkin's Labs
|
Lymph node biospy
Xray chest CT scan BM aspiration/biospy CBC, LDH, B2 microglobulin |
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Ann Arbor Staging
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I: One lymph node region
II: 2 or more lymph node regions on same side of diaphragm III: Lymph nodes on both sides of diaphragm IV: disseminated, BM involvement |
|
Normal lymph node microanatomy
|
Soft, gray-white, 1-2 cm nodules
Primary follicles made of B cells Pale staining germinal centers with maturing B cells Tingible body macrophages Germinal surrounded by eccentric darker area of unchallenged B mantle cells Interfollicular cortical cells = T cells Medulla = T cells and plasma cells |
|
Leukocyte Antigen
All leukocytes T Cells B Cells Granulocytes Monocytes Activation Blasts (immature) Lymphoblasts |
CD45
CD3, CD4, CD5, CD7, CD8 CD10, CD19, CD20, CD23 CD13, CD33, CD15 CD11c, CD14, CD64 CD30 CD34 TdT |
|
Precursor B-ALL/Lymphoblastic Lymphoma
Clinical |
-children
-fatigue, bleeding, infections |
|
Precursors B-ALL/Lymphoblastic Lymphoma
Morphology |
-BM and PB involvement
-CNS, liver, spleen, gonads -Thrombocytopenia, variable WBC count -20%+ blasts in blood or BM -Small to large blasts with variable nuclear/cytoplasmic ratio, fine chromatin, prominent nucleoli -No Auer rods -Hypercellular BM, packed with lymphoblasts |
|
Precursos B-ALL/Lymphoblastic Lymphoma Immunophenotype
|
TdT, CD10, CD19, CD20, CD34+
CD5, Ig- |
|
Precursor B-ALL/Lymphoblastic Lymphoma Cytogenetics
|
-good prognosis t(12;21), hyperdiploidy
-bad prognosis t(9;22), hypodiploidy |
|
Precursor T-Cell Lymphoblastic Lymphoma/ALL
Morphology |
-BM involvement, peripheral blood, CSF
-diffuse effacement by relatively small cells with very high nuclear/cytoplasmic ratio -no nucleoli -high mitotic rate |
|
Precursor T-Cell Lymphoblastic Lymphoma/ALL Immunophenotype
|
CD34, CD3, CD7, CD10+
CD4, CD8, TdT - |
|
Precursor T-Cell Lymphoblastic Lymphoma/ALL Cytogenetics
|
t(9;22)
|
|
Small Cell Lymphocytic Lymphoma Features
|
-lymphocytosis, lymph node involvement, elderly, indolent, chemo resistant
|
|
Small Cell Lymphocytic Lymphoma Immunophenotype
|
CD5, CD19, CD20+
CD10- |
|
Small Cell Lymphocytic Lymphoma Morphology
|
-Node effacement with diffuse sheets of small, mature, normal-appearing lymphocytes
-Proliferation centers |
|
Chronic Lymphocytic Lymphoma Features
|
-lymphocytosis, lymph node involvement
-elderly -indolent, chemo resistant |
|
Chronic Lymphocytic Lymphoma Immunophenotype
|
CD5, CD19, CD20+
CD10- |
|
Chronic Lymphocytic Lymphoma Morphology
|
PB = lymphocytosis with monomorphic population of small normal appearing lymphocytes, smudge cells
-dominate BM aspirate |
|
Follicular Lymphoma Immunophenotype
|
CD5-, CD10+, CD19+, surface light chain restriction
|
|
Follicular Lymphoma Morphology
|
germinal center B cells
-Monotonous lymphoma follicles with germinal centers efface normal architecture Centrocytes (small) and centroblasts (large) |
|
Follicular Lymphoma Cytogenetics
|
t(14;18)
bcl2 over-expression, inhibits apoptosis |
|
Follicular Lymphoma Clinical
|
Genearlized LAD and BM involvement
Indolent 7-9 year survival, chemo, resistant |
|
Mantle Cell Lymphoma Immunophenotype
|
CD5+, CD19+, CD20+, surface light chain restriction, cyclin D1+, CD10-, CD23-
|
|
Mantle Cell Lymphoma Cytogenetics
|
t(11;14) --> overexpression of cyclin D1 --> promotes S phase of cell cycle
|
|
Mantle Cell Lymphoma Morphology
|
B cell tumors of mantle cell origin
-expansion of mantle zone around a small germinal center or diffuse effacement -small cells with irregular nuclei |
|
Mantle Cell Lymphoma Clinical
|
-LAD
-50% spleen involvement, extranodal disease -aggressive, 3-4 year survival -chemo resistant |
|
Diffuse Large B Cell Lymphoma Morphology
|
-broad age range
-diffuse proliferation of large cells -vesicular chromatin -irregular nuclear borders -large nucleoli |
|
Diffuse Large B Cell Lymphoma Immunophenotype
|
CD19+, CD20+, CD5-, CD10+/-, surface light chain restriction
|
|
Diffuse Large B Cell Lymphoma Cytogenetics
|
t(3q27) t(14;18)
|
|
Diffuse Lrage B Cell Lymphoma Clinical
|
-large localized mass, extranodal sites
-HIV, EBV infectin -rapidly fatal -chemo responsive |
|
Burkitt Lymphoma
|
-responds well to chemo
-endemic, sporadic, and HIV-related |
|
Burkitt Lymphoma Immunophenotype
|
CD10+, CD19+, CD20+, CD5-, TdT-, surface light chain restriction
|
|
Burkitt Lymphoma Cytogenetics
|
c-MYC oncogene on C8
t(8;14) t(2;8) t(8;22) |
|
Burkitt Lymphoma Morphology
|
diffuse sheets of intermediate sized cells with vesicular chromatin
mutliple small nucleoli moderate amount of basophilic cytoplasm numerous mitotic figures numerous tingible body macrophages "starry sky" |
|
Marginal Zone Lymphoma
|
Mucosal associated lymphoid tissues, spleen, nodes
Chronic inflammation Lymphoepithelial lesions Splenomegaly, pancytopenia |
|
Hairy Cell Leukemia
|
-red pulp of spleen and BM, mild peripherilization, rare node involvement
-middle aged adults, males |
|
Hairy Cell Leukemia Immunophenotype
|
CD19+, CD20+, CD11c+, CD103+, TRAP+
|
|
Hairy Cell Leukemia Morphology
|
PB: lymphocytes with fine chromatin, veniform nuclei, hairy projections
Spleen: red pulp infiltration, white pulp obliteration BM: variably diffuse infiltrate of fried egg cells, myelofibrosis |
|
Plasma Cell Neoplasms
|
monoclonal Ig-secreting plasma cells
rouleaux formation (parproteins make RBCs stick together) |
|
Solitary Plasmacytoma
|
plasma cells secrete monoclonal Ig
|
|
Primary Amyloidosis
|
amyloid made of free light chains (bence-jones proteins)
|
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MGUS
|
asymptomatic paraproteinemia, elderly
|
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Multiple Myeloma
|
multifocal bone involvement with malignant plasma cells that secrete monoclonal Ig
50-60 y/o |
|
Multiple Myeloma Clinical
|
multifocal bone lesions
vertebral collapse, back/neck pain, generalized osteopenia, hypercalcemia -free light chains (Bence-Jones proteins) may cause renal failure and/or amyloidosis predispoistion to bacterial infections |
|
Multiple Myeloma Morphology
|
BM always involved, nodes rarely
Increase in plasma cells (>30% of BM) Fine chromatin, prominent nucleoli (no clock face), multinucleated |
|
Multiple Myeloma Immunophenotype
|
CD38+, CD138+, CD56+, cytoplasmic light chain restriction, IL-6, RANKL
|
|
Adult T-Cell Leukemia/Lymphoma Virus
|
HTLV-1
|
|
Multiple Myeloma Clinical
|
multifocal bone lesions
vertebral collapse, back/neck pain, generalized osteopenia, hypercalcemia -free light chains (Bence-Jones proteins) may cause renal failure and/or amyloidosis predispoistion to bacterial infections |
|
Adult T-Cell Leukemia/Lymphoma Clinical
|
skin lesions
generalized LAD hepatosplenomegaly lymphocytosis hypercalcemia rapidly progressive, death within 1 year |
|
Multiple Myeloma Morphology
|
BM always involved, nodes rarely
Increase in plasma cells (>30% of BM) Fine chromatin, prominent nucleoli (no clock face), multinucleated |
|
Multiple Myeloma Clinical
|
multifocal bone lesions
vertebral collapse, back/neck pain, generalized osteopenia, hypercalcemia -free light chains (Bence-Jones proteins) may cause renal failure and/or amyloidosis predispoistion to bacterial infections |
|
Mycosis Fungoides
|
premycotic, plaque, tumor phases
neoplastic T cells with cerebriform nuclei infiltrate upper dermis and epidermis can involve nodes, BM indolent |
|
Multiple Myeloma Morphology
|
BM always involved, nodes rarely
Increase in plasma cells (>30% of BM) Fine chromatin, prominent nucleoli (no clock face), multinucleated |
|
Sezary Syndrome
|
a variant of mycosis fungoides with generalized exfoliative erythroderma and periphelaziation of malignant T cells
|
|
Multiple Myeloma Immunophenotype
|
CD38+, CD138+, CD56+, cytoplasmic light chain restriction, IL-6, RANKL
|
|
Anaplastic Large Lymphoma Immunophenotype
|
CD30+, T cell, Null Cell
|
|
Adult T-Cell Leukemia/Lymphoma Virus
|
HTLV-1
|
|
Anaplastic Large Lymphoma Cytogenetics
|
ALK gene of 2p23 rearrangement
|
|
Multiple Myeloma Immunophenotype
|
CD38+, CD138+, CD56+, cytoplasmic light chain restriction, IL-6, RANKL
|
|
Adult T-Cell Leukemia/Lymphoma Virus
|
HTLV-1
|
|
Adult T-Cell Leukemia/Lymphoma Clinical
|
skin lesions
generalized LAD hepatosplenomegaly lymphocytosis hypercalcemia rapidly progressive, death within 1 year |
|
Adult T-Cell Leukemia/Lymphoma Clinical
|
skin lesions
generalized LAD hepatosplenomegaly lymphocytosis hypercalcemia rapidly progressive, death within 1 year |
|
Mycosis Fungoides
|
premycotic, plaque, tumor phases
neoplastic T cells with cerebriform nuclei infiltrate upper dermis and epidermis can involve nodes, BM indolent |
|
Anaplastic Large Lymphoma Morphology
|
sinusoidal spread within node leading to diffuse effacement with large cells with very anaplastic nuclei (hallmark cells) and many mitotic figures
mixed inflammatory infiltrate can cause confusion with Hodgkins |
|
Sezary Syndrome
|
a variant of mycosis fungoides with generalized exfoliative erythroderma and periphelaziation of malignant T cells
|
|
Hodgkin Lymphoma
|
Spread to contiguous nodes
Extranodal areas rarely involved Few malignant cells (RS cells) in inflammatory background Adolescents, younga dults, elderly |
|
Mycosis Fungoides
|
premycotic, plaque, tumor phases
neoplastic T cells with cerebriform nuclei infiltrate upper dermis and epidermis can involve nodes, BM indolent |
|
Anaplastic Large Lymphoma Immunophenotype
|
CD30+, T cell, Null Cell
|
|
Reed-Sternberg Cells
|
malignant cells in HL
probably of B cell origin |
|
Sezary Syndrome
|
a variant of mycosis fungoides with generalized exfoliative erythroderma and periphelaziation of malignant T cells
|
|
Anaplastic Large Lymphoma Cytogenetics
|
ALK gene of 2p23 rearrangement
|
|
Diagnostic RS Cells
|
Very large, binucleated
Huge nucleoli in each nucleus |
|
Anaplastic Large Lymphoma Morphology
|
sinusoidal spread within node leading to diffuse effacement with large cells with very anaplastic nuclei (hallmark cells) and many mitotic figures
mixed inflammatory infiltrate can cause confusion with Hodgkins |
|
Variant RS Cells
|
Mononucleated
|
|
Hodgkin Lymphoma
|
Spread to contiguous nodes
Extranodal areas rarely involved Few malignant cells (RS cells) in inflammatory background Adolescents, younga dults, elderly |
|
Mummy Cells
|
degenerated, pyknotic RS cells
|
|
Reed-Sternberg Cells
|
malignant cells in HL
probably of B cell origin |
|
Anaplastic Large Lymphoma Immunophenotype
|
CD30+, T cell, Null Cell
|
|
Diagnostic RS Cells
|
Very large, binucleated
Huge nucleoli in each nucleus |
|
Anaplastic Large Lymphoma Cytogenetics
|
ALK gene of 2p23 rearrangement
|
|
Anaplastic Large Lymphoma Morphology
|
sinusoidal spread within node leading to diffuse effacement with large cells with very anaplastic nuclei (hallmark cells) and many mitotic figures
mixed inflammatory infiltrate can cause confusion with Hodgkins |
|
Variant RS Cells
|
Mononucleated
|
|
Hodgkin Lymphoma
|
Spread to contiguous nodes
Extranodal areas rarely involved Few malignant cells (RS cells) in inflammatory background Adolescents, younga dults, elderly |
|
Mummy Cells
|
degenerated, pyknotic RS cells
|
|
Reed-Sternberg Cells
|
malignant cells in HL
probably of B cell origin |
|
Diagnostic RS Cells
|
Very large, binucleated
Huge nucleoli in each nucleus |
|
Variant RS Cells
|
Mononucleated
|
|
Mummy Cells
|
degenerated, pyknotic RS cells
|
|
Multiple Myeloma Clinical
|
multifocal bone lesions
vertebral collapse, back/neck pain, generalized osteopenia, hypercalcemia -free light chains (Bence-Jones proteins) may cause renal failure and/or amyloidosis predispoistion to bacterial infections |
|
Multiple Myeloma Morphology
|
BM always involved, nodes rarely
Increase in plasma cells (>30% of BM) Fine chromatin, prominent nucleoli (no clock face), multinucleated |
|
Multiple Myeloma Immunophenotype
|
CD38+, CD138+, CD56+, cytoplasmic light chain restriction, IL-6, RANKL
|
|
Adult T-Cell Leukemia/Lymphoma Virus
|
HTLV-1
|
|
Adult T-Cell Leukemia/Lymphoma Clinical
|
skin lesions
generalized LAD hepatosplenomegaly lymphocytosis hypercalcemia rapidly progressive, death within 1 year |
|
Mycosis Fungoides
|
premycotic, plaque, tumor phases
neoplastic T cells with cerebriform nuclei infiltrate upper dermis and epidermis can involve nodes, BM indolent |
|
Sezary Syndrome
|
a variant of mycosis fungoides with generalized exfoliative erythroderma and periphelaziation of malignant T cells
|
|
Anaplastic Large Lymphoma Immunophenotype
|
CD30+, T cell, Null Cell
|
|
Anaplastic Large Lymphoma Cytogenetics
|
ALK gene of 2p23 rearrangement
|
|
Anaplastic Large Lymphoma Morphology
|
sinusoidal spread within node leading to diffuse effacement with large cells with very anaplastic nuclei (hallmark cells) and many mitotic figures
mixed inflammatory infiltrate can cause confusion with Hodgkins |
|
Hodgkin Lymphoma
|
Spread to contiguous nodes
Extranodal areas rarely involved Few malignant cells (RS cells) in inflammatory background Adolescents, younga dults, elderly |
|
Reed-Sternberg Cells
|
malignant cells in HL
probably of B cell origin |
|
Diagnostic RS Cells
|
Very large, binucleated
Huge nucleoli in each nucleus |
|
Variant RS Cells
|
Mononucleated
|
|
Mummy Cells
|
degenerated, pyknotic RS cells
|
|
Lymphocyte Predominant HL
|
CD20+, CD45+, CD15-, CD30-
numerous, reactive small mature lymphocytes and histiocytes somewhat nodular very few RS cells L&H popcorn cells: single nucleus with very irregular nuclear borders, nucleoli |
|
Nodular Sclerosis HL
|
CD15+, CD30+, CD20-, CD45-
collagen bands divide tumor into nodules lacunar cells in background of inflammatory cells scattered RS cells cervical or mediastinal nodes |
|
Lymphocyte Depletion HL
|
CD15+, CD30+, CD20-, CD45-
relatively abundant RS cells, fewer inflammatory cells associated with EBV elderly, HIV patients |
|
Mixed Cellularity HL
|
CD15+, CD30+, CD20-, CD45-
prominent infiltrate of plasma cells, eosinophils, histiocytes, lymphocytes plentiful RS cells associated with EBV |
|
Lymphocyte Rich HL
|
CD15+, CD30+, CD20-, CD45-
closely resemebles lymphocyte predominant HL not as nodular more RS cells |
|
Langerhans Cell Histiocytosis
|
CD1a+, S-100+
Birbeck granules Vesicular nuclei with grooves and folds |
|
Langerhans Cell Histiocytosis
Letterer-Siwe (multifocal multisystem) Multifocal unisystem Unisystem |
<2 years old
Older children, Hand-Schuller, Christian triad Adolescents and adults |
|
Common Causes of LAD
|
malignancy
hematologic (lymphoma, leukemia) metastasis immunologic diseases endocrine/lipid storage diseases infectious diseases |
|
Infectious Agents of LAD
|
Yersinia pestis
Chlamydia trachmoatis (L1-L3) Bartonella henselae Sporothrix schenckii |
|
Yersinia Pestis (morphology, virulence)
|
Gram-neg rod
YOPS (TTSS); F1, PAP |
|
Chlamydia trachomatis (morphology, virulence)
|
Gram-neg organism
Intracellular growth, failure of endcrine/lysosome fusion |
|
Bartonella hensalae (morphology, virulence)
|
Gram-neg rod
Pili |
|
Sporthrix schenckii (morphology, virulence)
|
Thermally dimorphic fungus
No virulence |
|
Yersinia pestis
|
Sylvatic cycle
Fleas, infected rodents Plague (bubonic, pneumonic, systemic) High morbiidty/mortality |
|
Chlamydia trachomatis
|
Serovars L1-L3
Sexual transmission Humans only Lymphogranuloma venereum Systemic Indolent to morbid |
|
Bartonella henselae
|
Children
Fall/Winter Animal scratch/kittens Catscratch disease Self-limiting Painful, regional LAD |
|
Sporothrix schenckii
|
N and S America, Japan
Soil and decaying vegetation Forestry, mining, gardening Sphagrum moss in floristry Local trauma Local with lymphatic spread but no dissemination Sporotrichosis |
|
Yersinia pestis (a case)
|
Plague
Inguinal LAD Sepsis, pulmonary infiltrate |
|
Yersinia pestis TX
|
Doxycyline for prophylaxis
Genatmicin |
|
Bubonic Plague
|
7 days after bite
High fever Painful bubo-femoral, ingunal, axillary Bacteremia |
|
Pneumonic Plague
|
2-3 days after inhalation
Fever, malaise, pulmonary signs Person to person spread |
|
Yersinia Pestis Diagnosis
|
CBC
Urinalysis Arterial blood gas Culture Chest x-ray Bipolar staining rods (safety pins) |
|
Yersinia Pestis course
|
Shock
Sepsis Acute renal failure ARDS DIC |
|
Lymphogranuloma venereum (a case)
|
tender inguinal LAD
fever, chills, headache nodes enlarge, rupture, and form draining fistulas anogenital strictures systemic spread |
|
Lymphogranuloma venereum diagnosis
|
Microimmunofluorescence serology
PCR* |
|
Lymphogranuloma venereum TX
|
Tetracycline x 21 days
|
|
Catscratch disease (a case)
|
papulopustular lesions
LAD of head, neck, upper limb nodes Fever, headache, fatigue, anorexia |
|
Catscratch disease DDx
|
Mono
TB Lymphoma |
|
Catscratch disease Diagnosis
|
DFA
|
|
Sporotrichosis (a case)
|
Trees
lesions on hands, fingers Relatively painless Lesions spread up arm |
|
Sporotrichosis Pathology/DX
|
Sporothrix schenckii
thermally dimorphic fungus tissue sections |
|
Sporotrichosis DDx
|
Squamous cell carcinoma
Mycobacterial and nocardial infection |
|
Nonthrombocytopenic purpuras
|
-increased vascular fragility
-bleeding is not usually serious -PT, PTT, PLT, bleeding time normal -petechiae, purpura, hemorrhage |
|
Acquired causes of nonthrombocytopenic purpuras
|
Infections (meningococcemia, infective endocarditis, rickettsioses)
Drugs (aspirin, atropine, quinine, Coumarin) Senile purpura (collagen atrophy) Scurvy (need vit c to make collagen) Cushings Syndrome (protein wasting) Amyloidosis Scleroderma Henoch-Schnolein purpura |
|
Inherited causes of nonthrombocytopenic pupuras
|
Ehlers-Danlos syndrome
Hereditary hemorrhagic telangiectasia Marfan's syndrome Osteogenesis Imperfecta Pseudoxanthoma elasticum |
|
Thrombocytopenia Causes
|
Defective DNA syntehsis/defective production
Decreased survival Sequestration (hypersplenism) Dilutional (massive transfusions) |
|
Idiopathic Thrombocytopenia Purpura
|
Decreased platelet survival
|
|
Idiopathic Thrombocytopenia Purpura Acute
|
Sudden onset
Children Post viral Self-limiting Steroid treatment |
|
Idiopathic Thrombocytopenia Purpura Chronic
|
Autoantibodies against IIb-IIIa or Ib-IX
Opsonize platelets --> phagocytosized Female:male 3:1 Petechiae and ecchymoses Treat with corticosteroids Splenectomy maybe |
|
Idiopathic Thrombocytopenia Purpura Labs
|
Decreased PLT
Normal or Increased megs in BM Large platelets Increased bleeding time Normal PT, PTT |
|
Heparin-induced thrombocytopenia
|
Antibody recognizes heparin bound to PF4
Decreased PLT within 10 days/or hours Can develop thrombosis |
|
HIV-associated thrombocytopenia
|
Impaired production and increased destruction
Cells prone to apoptosis Autoantibody against gp IIb-IIIa Immune complexes Tx with AZT or alpha-interferon |
|
Thrombotic thrombocytopenic purpura
|
Similar to ITP
|
|
Thrombotic thrombocytopenic purpura Labs
|
Decreased PLT
PT and PTT normal |
|
Thrombotic thrombocytopenic purpura Acquired
|
Drugs
Pregnancy SLE Infection Malignancy |
|
Thrombotic thrombocytopenic purpura Inherited
|
Lack of vWF cleaving enzyme --> accumulation of ULvWF --> microthrombi
|
|
Congenital Platelet Formation Disorders
|
Bernard-Soulier syndrome (adhesion defect, Gp 1b-IX)
Glanzmann's thrombathenia (Gp IIb-IIIa aggregration defects) Gray platelet syndrome, Delta, Chediak-Higashi, Wiskoff-Aldrich (storage pool disorders-platelet secretion defects) |
|
Acquired Platelet Function Disorders
|
Drug induced
Uremia Liver disease (increased FDPs) Paraneoplastic platelet dysfunction |
|
Hemophilia A
|
Factor VIII deficiency
Most common hereditary bleeding disorder X-linked recessive |
|
Hemophilia A clincal
|
Easy bruising
Massive hemorrhage after trauma/surgery Spontaneous bleeding into joints (hemarthroses) and soft tissue |
|
Hemophilia A Labs
|
Increased PTT
Decreased Factor VIII Normal vWF and ristocetin cofactor activity Normal bleeding time |
|
Hemophilia A Treatment
|
NO aspirin
Demsopressin before procedures Factor VIII replacement (FFP, cryoprecipitate, Factor VIII concentrates, recombinant F8) |
|
Hemophilia B (Christmas disease)
|
Factor IX deficiency
Same clinically as Hemophilia A X-linked recessive |
|
Hemophilia B Labs
|
Increased PTT
Decreased Factor IX Normal bleeding time Normal thrombin time |
|
Hemophilia B Treatment
|
NO aspirin
Factor IX concentrate Prothrombin complex Activated prothrombin complex FFP |
|
Von Willebrand's Disease
|
Most common inherited
Glycoproteins vWF: facilitates platelet adhesion to vessel wall, plasma carrier and stabilizer for FVIII |
|
Von Willebrand's Disease Type I
|
Increased PTT
Autosomal dominant Excessive bleeding after surgery/trauma |
|
Von Willebrand's Disease Type II
|
Autosomal dominant
Dysfunction vWF (but normal levels) |
|
Von Willebrand's Disease Type III
|
Increased PTT
Autosomal recessive |
|
Von Willebrand's Disease Labs
|
Normal PLT
Increased bleeding time Decreased ristocetin cofactor activity Increased PTT in Type I and III |
|
Disseminated Intravascular Coagulopathy (DIC)
|
increased release of thrombin into circulation
fibrinolysis clotting factors are consumed |
|
Disseminated Intravascular Coagulopathy (DIC) Triggers
|
Release of tissue factor or thromboplsatic substances
Widespread endothelial damage |
|
Disseminated Intravascular Coagulopathy (DIC) Causes
|
Pathologic activation of extrinsic and/or intrinsic pathways or impaired clot inhibitory influences
|
|
Disseminated Intravascular Coagulopathy (DIC) Acute (causes)
|
Infection
Obsteric Complications Trauma Transfusion Liver disease |
|
Disseminated Intravascular Coagulopathy (DIC) Chronic (causes)
|
Malignancy
Retained dead fetus Liver disease Severe localized IC |
|
Disseminated Intravascular Coagulopathy (DIC) Clinical
|
Microangiopathic hemolytic anemia
Dyspnea Cyanosis Respiratory failure Convulsions Oliguria Acute renal failure Progressive circulatory failure Shock |
|
Disseminated Intravascular Coagulopathy (DIC) Treatment
|
REMOVE CAUSE
|
|
Hereditary Hypercoagulative States
|
Protein C deficiency
Protein S deficiency Antithrombin III deficiency Factor V Leiden -must be considered in patients <50 y/o who present with thrombosis in absence of any acquired cause |
|
Acquired Hypercoagulative States
|
Antiphospholipid Antibody Syndromes
-anticardiolipin -lupus-like anticoagulants -present with recurrent venous or arterial thrombi, repeated miscarriage, cardiac valve vegetattions or thrombocytopenia |