Exam 1 Pathology Flashcards
Layers of the Epidermis
Stratum… Corneum, Lucidum, Granulosum, Spinosum, Basale
Come, Let’s Get Sun Burned
Acanthosis vs papillomatosis
acanthosis: epidermal hyperplasia
papillomatosis: dermal papilla enlargement and hyperplasia
Lentigo
flat lesion
melanocyte hyperplasia
suborrheic keratoses
Flat, greasy, pigmented squamous epithelial proliferation
Benign tumor in older patients
Leser-Trelat sign: rapid onset of multiple keratoses may indicates possible malignancy (GI adenocarcinoma)
acanthosis nigricans
80% benign: diabetes, pitutary/pineal tumor
20% associated with malignacy (GI adenocarcinoma)
Acrochordon
skin tag
actinic keratosis
Premalignant skin lesion associated with sun exposure
rough brown plaques
may be horn like
benign fibrous histiocytoma (dermatofibroma)
painless scar-like lesion leftover from trauma
dermatofibrosarcoma protuberans
overexpression of PDGF-B (platelet derived growth factor)
translocation of COL1A1 (collagen) onto the PDGF gene causes upregulation
wheals
hives, aka uticaria
Eczema
atopic dermatitis
allergic skin contact
Erythema multiforme
hypersensitivity in response to infections (mycoplasma, HSV) or drugs (sulfa drugs, B-lactams, phenytoin)
Target lesions
Psoriasis
silver scaly lesions, nail pitting
+ Auspitz sign → bleed when picked
Seborrheic dermatitis
occurs in areas rich with sebaceous glands, (scalp, face, periocular, ear)
possibly caused by fungus called Malassezia.
correlated with Parkinsons and HIV
Lichen Planus
Pruritic, Purple, Polygonal, Planar Papules, and plaques
with Wickham striae and sawtooth pattern at the dermal-epidermal junction
resolve spontaneously
Pemphigus
IgG autoantibodies against desmoglein flaccid blisters (subcorneal or suprabasal) type 2 hypersensitivity disorder potentially fatal
Bullous pemphigoid
IgG autoantibodies against hemidesmosomes (epidermal basement membrane) tense blisters (subepidermal) type 2 hypersensitivity less severe than pemphigus
Dermatitis herpetiformis
IgA deposits
grouped pruritic blisters
associated with celiacs disease
Epidermolysis bullosa
defects in structural proteins
↑ blisters, trauma, rubbing
Porphyria cutana tarda
blistering in response to sunlight
Red marrow
hematopioetic
axial skeleton, hands and feet
yellow marrow
fat
long bones
Normal WBC ranges
5,000-10,000
Normal granuloocyte ranges
40-70%
Normal RBC ranges
3,500,000 - 5,000,000 (higher in men)
Normal platelet ranges
150,000 - 450,000
Most numerous white blood cell
neutrophil (40-80%)
Leukemia
Bone marrow cancer of WBCs causes…
marrow failure
↓ RBCs and mature WBCs
infections
hemorrhage (↓ platelets)
presents with circulating malignant WBCs
Metastasis to liver, spleen, lymph nodes, and skin possible
Acute Lymphoblastic leukemia/lymphoma (ALL)
Lymphoid neoplasm Children TdT+ (pre-T and pre-B cells) CD10+ (pre-B cells) t(12;21) → better prognosis May spread to CNS and testis T-cell ALL can present with mediastinal mass associated with Down Syndrome
Chonic lymphocytic leukemia/small lymphocytic lymphoma
lymphoid neoplasm (B-cell) age > 60 most common adult leukemia CD20+ CD23+ CD5+ smudge cells in peripheral blood smear CLL (crushed little lymphocytes) autoimmune hemolytic anemia Richter transformation → CLL/SLL can transform into an aggressive lymphoma, diffuse large B-cell lymphoma
TdT+
CD10+
ALL
CD20+
CD23+
CD5+
CLL/SLL
Richter transformation
CLL/SLL
smudge cells
CLL/SLL
Hairy cell leukemia
Lymphoid neoplasm
adult males
mature B-cell tumor
cells have filamentous hair-like projections
marrow fibrosis → massive splenomegaly and pancytopenia
TRAP+ stain
BRAF mutations
Acute myelogenous leukemia
Myeloid neoplasm
age ~ 65
Auer rods
↑↑↑ circulating myeloblasts on peripheral smear
Can arise of pre-existing myelodysplastic syndromes
exhibit anemia, neutropenia, thrombocytopenia
Chronic myelogenous leukemia
myeloid neoplasm age 45-85 Philadelphia chromosome (t[9;22], BCR-ABL) splenomegaly low LAP
Hodgkin vs NH lymphomas
Both present with low grade fever, night sweats, weight loss
Hodgkin: better prognosis localized group of nodes Reed sternberg cells EBV
NHL:
multiple lymph nodes, extranodal involvement
majority are B-cells
Autoimmune disease and EBV, HIV, HTLV
frequently involve waldeyer ring and mesenteric nodes
Hodgkin lymphomas and its subtypes
Reed sternberg cells (binucleate, owl eye cells that are CD15+ and CD30+)
subtypes:
Nodular sclerosis → most common
Lymphocyte rich → best prognosis
Mixed cellularity → eosinophilia, seen in the immunocompromised (EBV)
Lymphocyte depleted → seen in the immunocompromised (EBV)
Burkitt lymphoma
NHL
CHILDREN OR YOUNG ADULTS (all other NHL are adults)
t(8;14) translocation of c-MYC(8) and heavy chain Ig (14)
Starry sky appearance with interspersed tingible body macrophages
jaw lesions in Africa
pelvis or abdomen in sporadic form
Diffuse large B-cell lymphoma
NHL (most common one)
older adults (80%), children (20%)
BCL-2 and BCL-6 alterations
Follicular lymphoma
NHL
adults
t(14;18) translocation of heavy chain Ig (14) and BCL-2 (18)
painless waxing and waning lymphadenopathy
Mantle cell lymphoma
NHL
Adult males (MANtle)
t(11;14) translocation of cyclin D1 (11) and heavy chain Ig (14)
CD5+
very aggressive, patients present in late stages
Marginal cell lymphoma
NHL
adults
t(11;18) translocation of cyclin D1 (11) and BCL-2 (18)
associated with chronic inflammation (sjogrens, chronic gastritis)
Primary central nervous system lymphoma
NHL adults associated with HIV/EBV considered AIDs defining illness ring enhancing mass lesion on MRI of brain
Adult T-cell lymphoma
NHL of T-cells adults caused by HTLV (associated with IV drug abuse) present with cutaneous lesions common in Japan, Africa, and caribbean
Mycosis fungoides
NHL of T-cells
Adults
skin patches and plaques characterized by atypical CD4+ cells with cerebriform nuclei and intraepidermal neoplastic cell aggregates (Pautrier microabcesses)
may progress to Sezary syndrome (T-cell leukemia)
Sezary syndrome
T-cell leukemia
associated with mycosis fungoides
t(8;14)
Burkitt lymphoma (burk-8)
t(11;14)
mantle cell lymphoma
t(11;18)
marginal zone lymphoma
t(14;18)
follicular lymphoma
t(15;17)
APL (M3 type AML)
t(9;22)
Philadelphia chromosome
CML
Multiple myeloma
Plasma cell cancer in marrow produces large amounts of IgG or IgA ↑ susceptibility to infection Punched-out lytic bone lesions hypercalcemia and renal failure Ig light chains in urine (Bence jones protein) M spike on electrophoresis Rouleaux formation (RBC's stacked like poker chips)
Hypercalcemia
Renal failure
anemia
bone lytic lesions / back pain
Multiple myeloma
M spike on electrophoresis
multiple myeloma
Rouleaux formation
mutliple myeloma (RBCs stack up like poker chips)
Elevated serum Ig and Bence Jones protein in urine
multiple myeloma
monoclonal gammopathy of undetermined significance
Monoclonal expansion of plasma cells which may progress to multiple myeloma
asymptomatic and no other findings
Waldenstrom macroglobulinemia
hyperviscosity syndrome (blurred vision, Reynaud's) differentiate from multiple myeloma, no CRAB findings
myelodysplastic syndromes
Disorders involving ineffective hematopoiesis
Risk of transforming into AML
Refractory anemias…. (RA, RARS, RAEB…)
Polycythemia Vera
↑ in all Blood cell types, but RBCs in particular intense pruritus red-blue cyanosis DVT, stroke ↓ in EPO
Essential thrombocythemia
↑↑ in platelets and megakaryocytes
thrombosis
myelofibrosis
obliteration of bone marrow with fibrosis from non-neoplastic fibroblasts
massive splenomegaly
teardrop RBCs
Langerhans cell histiocytosis
Group of proliferative disorders
S-100 and CD1a + cells
Birbeck granules (tennis racket)
Presents in children as bony lytic lesions, with rash and recurrent otitis media
Birbeck granules
Langerhan cell histiocytosis
S-100 and CD1a +
Langerhan cell histiocytosis
Intravascular hemolysis vs Extravascular hemolysis
Intravascular → RBC destroyed in circulation
Extravascular → RBC destroyed in spleen or liver
intra → ↓haptoglobin, hemoglobinemia/uria
extra → spherocytes, splenomegaly
Hereditary spherocytosis
Intrinsic, extravascular anemia AD sphere-shaped RBCs eliminated in spleen splenomegaly ↑ MCHC (mean cell hemoglobin conc) ↓ MCV Many patients asymptomatic aplastic crisis can occur if infected with Parvovirus
G6PD deficiency
↑ RBC susceptibility to oxidant stress (infections, drugs, fava beans)
RBCs with Heinz bodies and bite cells
Hemoglobin C disease
Similar to Sickle Cell, but milder, confers same resistance to malaria
Distinctive Crystalline bars in RBCs
causes extravascular hemolysis
α-thalassemia
Microcytic, hypochromic anemia defect in α chain of hemoblobin gene deletion (vs ß which is due to point mutation)
(αα/α-) → minima (silent carrier)
(α-/α- ;trans) or (αα,– ;cis) → minor (mild anemia)
(–/-α) Hemoglobin H disease (moderate to severe anemia)
(–/–) Hemoglobin Barts disease (hydrops fetalis)
ß-thalassemia
Microcytic, hypochromic anemia
point mutation ↓ ß-globin synthesis
Heterozygote → minor (usually asymptomatic, ↑HbA)
Homozygote → major (severe anemia require transfusions, “Crew-cut” marrow expansion on skull x-ray)
Paroxysmal nocturnal hemoglobinuria
hemolytic anemia
Acquired mutation of PIGA
at risk for thrombosis and leukemia
Autoimmune hemolytic anemia
Warm (IgG) - chronic anemia seen in SLE, CLL, and certain drugs
Cold (IgM) - acute/chronic anemia triggered by cold seen in CLL, M. pneumoniae, Mononucleosis, painful blue fingers and toes
Both are Coombs +
Direct vs Indirect Coombs test
Direct → Pt RBCs
Indirect → Pt serum
Microangiopathic anemia
RBCs damaged when passing through obstructd or narrowed vessel
Schistocytes (helmet cells) on blood smear
megaloblastic anemia
Impaired DNA synthesis
includes: folate deficiency, Vit B12 deficiency, orotic aciduria
Vit B12 defic
causes megaloblastic anemia with neurologic symptoms
↑ homocysteine, methylmalonic acid
Pernicious anemia
megaloblastic anemia caused by autoimmune gastritis that impairs intrinsic factor production → vit B12 deficiency
Folate deficiency
megaloblastic anemia with no neurologic symptoms
↑ homocysteine
Iron deficiency
Microcytic, hypochromic anemia ↓ iron ↓ ferritin (iron stores) ↑ TIBC (total iron binding capacity) ↑ RDW (red cell distribution width)
Anemia of chronic disease
Normocytic anemia Chronic infection Chronic autoimmune disease Cancer iron is not released from tissue to be used, nor absorbed in the gut
↓ iron
↓ TIBC
↑ ferritin
Aplastic anemia
Normocytic anemia destruction or failure of hematopoietic cells due to... Drugs Virus (EBV, HIV, hepatitis) Fanconi syn Idiopathic (65%)
hypocellular marrow
Coagulation cascade
Intrinsic pathway: Factor 12, 11, 9, (10)
Extrinsic pathway: Factor 7
Common pathway: Factor 10, 13
PTT vs PT
PTT → intrinsic and common (monitors heparin)
PT → extrinsic and common (monitors coumadin)
Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome
Triad of thrombocytopenia, hemolytic anemia, and AKI
TTP → addition of neuro symptoms and fever
HUS → addition of bloody diarrhea
TTP → ↓ADAMTTS13
HUS → E.Coli (EHEC)
Bernard-Soulier syndrome
Platelet disorder, defect in adhesion
Glanzmann thrombasthenia
Platelet disorder, defect in aggregation
von Willebrand disease
Intrinsic pathway defect (↑ PTT)
Platelet plug formation defect
Ristocetin assay will cause no platelet aggregation
