Session 56-61

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Leukemia: Neoplastic expansion of hematopoietic cells in blood and bone marrow
Anemia, infection, bleeding
Acute Leukemia: expansion of immature cells
Chronic Leukemia: expansion of matture/differentiated cells
Meylogenous Leukemia: From Myeloid precursors
Lymphocytes Leukemia: From lymphoid precursors
Leukemia Etiology: Radiation, chemicals, hereditary
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
Meylodysplastic Syndrome: -"pre-leukemia"
defective hematopoiesis
elderly
dyspoiesis
<20% blasts in BM
progress to AML
Due to idiopathic or radiation or chemo
Pancytopenia: Decrease in all 3 cell lineages
Causes: aplastic anemia, drugs (chemo), viral (HCV, HIV, CMV), leukemia, vit B12 deficiency, tumor in BM
Multiple Myeloma: multifocal bone involvement with malignant plasma cells that secrete monoclonal Ig
50-60 y/o
Polycythemia: increased concentration of RBC mass, HGB, HCT
secondary to chronic smoking, COPD, high altitude
Polycythemia Vera: increased RBC mass
hyperviscosity, thrombosis
dizziness, headache, weakness
splenomegaly
phlebotomy, anti-coagulant, chemo
Thrombocythemia: Increase in number of PLT (>150,000 - 400,000)
Iron deficiency anemia, chronic infection, inflammation
Lymphoma: neoplastic expansion of lymphoid cells in lymph nodes presenting as a discrete tumor mass
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
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
Ann Arbor Staging: 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)
MGUS: asymptomatic paraproteinemia, elderly
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

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