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122 Cards in this Set
- Front
- Back
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erythrocyte |
Carries O2 to tissue, CO2 to lungs large SA:V ratio life span: 120 days needs glucose membrane: Cl-/HCO3- antiporter
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anisocytosis |
varying sizes
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poikilocytosis |
varying shapes
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reticulocyte |
immature RBC; reflects erythroid proliferation |
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thrombocyte |
platelet derived from megakaryocytes lifespan: 8-10 days interacts with fibrinogen to form platelet plug contains ADP, Ca2+ granules alpha granules: vWF, fibrinogen 1/3 platelet pool stored in spleen |
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GPIb |
vWF receptor
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GpIIb/IIIa |
fibrinogen receptor |
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granulocytes |
PMN eosinophil basophil
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mononuclear cells |
monocytes lymphocytes |
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normal WBC count |
4k-10k |
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WBC differential from highest to lowest (Neutrophils Like Making Everything Better) |
Neutrophils Lymphocytes Monocytes Eosinophils Basophils
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Neutrophil |
PMN acute inflammatory response cel bacterial infections phagocytic multilobed nucleus granules
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hypersegmented PMNs (5 or more lobes) |
vit B12/folate deficiency
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increased band cells (immature PMNs) |
states of myeloid proliferation (bacterial infections, CML) |
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PMN chemotactic factors |
C5a, IL-8, kallikrein, PAF |
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Monocyte |
differentiates into macrophage in tisues large, kidney-shaped nucleus "frosted glass" cytoplasm (in blood) |
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macrophage |
Phagocytosis long life in tissues differentiate from circulating blood monocytes activated by gamma interferon can function as APC via MHC II
important component of granuloma formation Lipid A from bacterial LPS binds CD14 on macrophages to initiate septic shock |
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Eosinophil |
Defends against helminthic infections bilobate nucleus larged eosinophilic granules phagocytose antigen-antibody complexes histaminase, major basic protein
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Causes of eosinophilia (NAACP) |
Neoplasia Asthma Allergic processes Chronic adrenal insufficiency Parasites |
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Basophil |
mediates allergic reaction densely basophilic granules contain heparin, histamine leukotrienes synthesized on demand |
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Basophilia uncommon, but can be indicator of... |
myeloproliferative disease, particularly CML
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Mast cell |
mediates allergic reaction in local tissue contains basophilic granules Bind the Fc portion of IgE to membrane IgE cross-links upon antigen binding, causing degranulation, which releases histamine, heparin, and eosinophil chemotactic factors
Involved in type I hypersensitivity rxns Cromolyn sodium prevents mast cell degranulation (asthma prophylaxis) |
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Dendritic cell |
Highly phagocytic APC link between innate and adaptive immune system expresses MHC Class II and Fc receptors on surface Langerhans cell of skin |
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Lymphocyte |
B cells, T cells, NK cells B and T cells mediate adaptive immunity NK cells: innate immune response densely-staining nucleus, small amount of pale cytoplasm |
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B cell |
Part of humoral immune response originates from stem cell in bone marrow and matures in marrow Migrates to peripheral lymphoid tissue When antigen is encountered, B cells differentiate into plasma cells (produce ABs), memory cells Can function as APC via MHC II
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T cell |
Mediates cellular immune response originates from stem cells in bone marrow, but maturesin thymus T cells differentiate into cytotoxic T cells (CD8, recognize MHC I), helper T cells (CD4, recognize MHC II), regulator T cells. CD28 necessary for T cell activation. Majority of circulating lymphocytes are T cells (80%). |
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Plasma cell |
Produces large amounts of antibody specific to a particular antigen. "Clock face" chromatin distribution abundant RER, well-developed Golgi Multiple myeloma is a plasma cell cancer |
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Acanthocyte spur cell |
liver disease |
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basophilic stippling |
lead poisoning |
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degmacyte ("bite cell") |
G6PD deficiency |
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elliptocyte |
hereditary elliptocytosis |
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macro-ovalocyte |
megaloblastic anemia (also hypersegmented PMNs) marrow failure |
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ringed sideroblast |
sideroblastic anemia excess iron in mitochondria |
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schistocyte (helmet cell) |
DIC TTY/HUS HELLP mechanical hemolysis (heart valve prosthesis) |
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Sickle cell |
sickle cell anemia sickling occurs with dehydration, deoxygenation, at high altitude |
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spherocyte |
hereditary spherocytosis drug and infection-induced hemolytic anemia |
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Dacrocyte (teardrop cell) |
Bone marrow infiltration RBC "sheds a tear" because it's mechanically squeezed out of its home in the bone marrow |
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Target Cell |
HALT HbC disease Asplenia Liver disease Thalassemia "HALT", said the hunter to his target |
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Heinz bodies |
oxidation of Hb-SH groups to disulfide bonds--> Hb precipitation with subsequent phagocytic damage to RBC membrane--> bite cells
Seen in G6PD deficiency; Heinz-body like inclusions seen in alpha thal |
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Howell-Jolly bodies |
Basophilic nuclear remnants found in RBCs Howell-Jolly bodies are normally removed from RBCs by splenic macrophages
Seen in patients with functional hyposplenia or asplenia |
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Iron deficiency findings (iron, TIBC, ferritin) |
low Fe hi TIBC low Ferritin
koilonychia microcytosis, hypochromia |
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Plummer Vinson syndrom |
triad of iron deficiency anemia, esophageal webs, atrophic glossitis |
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alpha thal: cis detions |
Asians |
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alpha thal: trans deletions |
African |
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y4 (gamma 4) |
Hb Barts-- incompatible with life-- hydrops fetalis |
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HbH |
3 allele deletion--> beta4 |
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Beta thal minor |
heterozygote beta chain underproduced usually asx dx confirmed by increase HbA2 on electrophoresis |
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Beta thal major |
homozygous beta chain absent-->severe anemia requiring blood transfusions (risk of secondary hemochromatosis) marrow expansion ("crew cut" on skull x ray) skeletal deformities, chipmunk facies extramedullary hematopoiesis
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HbF |
alpha2gamma2 protective in infant vs. beta thal major |
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HbS/Beta thal het |
mild to moderate sickle cell disease depending on amount of beta globin production |
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lead poisoning |
increased RBC protoporhyrin LEAD: Lead lines on gingivae and on metaphyses of long bones Encephalopathy and erythrocyte basophilic stippling Drops: wrist and foot drop Dimercaprol and EDTA are 1st line rx Succimer used for Chelation for all kids (sucks to be a kid who eats lead0 |
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Sideroblastic anemia |
defect in heme synthesis genetic, acquired, reversible causes ringed sideroblasts (iron-laden, Prussian-blue stained mitochondria seen in bone marrow) increased FE, decreased TIBC, increased ferritin rx: pyridoxine (B6) |
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macrocytic anemias |
megaloblastic anemia, nonmegaloblastic anemia |
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megaloblastic anemia |
impaired DNA synthesis--> maturation of nucleus of precursor cells in bone marrow delayed relative to maturation in cytoplasm
findings: RBC macrocytosis, hypersegmented PMNs, glossitis |
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Folate deficiency |
causes: malnutrition, malabsorption, drugs, increased requirement (hemolytic anemia, pregnancy)
findings: increased homocysteine, normal methylmalonic acid no neurologic sx (vs B12 deficiency) |
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B12 (cobalamin) deficiency |
causes: insufficient intake (veganism), malabsorption (Crohn disease), pernicious anemia, Diphyllobothrium latum (fish tapeworm), gastrectomy
findings: increased homocysteine, increased methylmalonic acid neurologic sx: subacute combined degeneration (B12 in FA, myelin synthesis pathway0 |
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orotic aciduria
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inability to convert orotic acid to UMP because of defect in UMP synthase AR |
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nonmegaloblastic macrocytic anemia |
macrocytic anemia in which DNA synthesis is unimpaired
causes: alcoholism, liver disease, hypothyroidism, reticulocytosis
findings: RBC macrocytosis wihtout hypersegmented PMNs |
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normocytic, normochromic anemia |
nonhemolytic hemolytic
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intravascular hemolysis |
findings: decreased haptoglobin, increased LDH, schistocytes, increased reticulocytes on blood smear hemoglobinuria, hemosiderinuria and urobilinogen in urine
mechanical hemolysis, paroxysmal nocturnal hemoglobinuria, MAHA |
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extravascular hemolysis |
macrophages in spleen clear RBCs. Spherocytes in peripheral smear increased LDH no hemoglobinuria/hemosiderinuria increased unconjugated bilirubin |
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Anemia of chronic disease |
inflammation--> increased hepcidin (inhibits iron transport)-->decreased release of iron from macrophages
rhematoid arthritis, SLE, neoplastic disorders, CKD
findings: decreased iron, decreased TIBC, increased ferritin
rx: EPO (CKD only) |
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aplastic anemia |
Caused by failure or destruction of myeloid stem cells due to radiation/drugs, virla agents, fanconi anemia, idiopathic
pnacytopenia, severe anemia, leukopenia, thrombocytopenia
hypocellular bone marrow with fatty infiltration
fatigue, malaise, pallor, petechia, infection
rx: withdrawal of offending agent, immunouppression |
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Hereditary spherocytosis |
Defect in proteins interacting with RBC membrane skeleton and plasma membrane
small, round RBCs, less SA, no central pallor
increased MCHC, increased RDW premature removal by spleen
findings: splenomegaly, aplastic crisis labs: osmotic fragility test positive, normal to decreased MCV
rx: splenectomy |
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G6PD deficiency |
most common enzymatic disorder of RBCs XLR Defect in G6PD--> reduced glutathione--> increased RBC susceptibility to oxidant stress
hemolytic anemia following oxidative stress (sulfa drugs, fava beans)
findings: back pain, hemoglobinuria after oxidant stress Lab: smear shows Heinz bodies, bite cells
"Stress makes me eat bites of fava beans with Heinz ketchup" |
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Pryruvate kinase deficiency |
Glutamic acid to lysine mutation in beta globin |
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paroxysmal nocturnal hemoglobinuria |
increased complement-mediated RBC lysis acquired mutation in hematopoietic stem cell increased incidenceof acute leukemias
Triad: Coombs negative hemolytic anemia pancytopenia VT
rx: eculizumab |
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Sickle cell anemia |
HbS point mutation AA replacement in B chain (glutamic acid substituted with valine)
low O2, hi altitude precipitates sickling
newborns initially asx due to HbF protection
crew cut on skull x ray erythropoiesis |
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AIHA |
Warm and cold AIHA usually coombs positive |
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Warm agglutinin |
IgG SLE, CLL warm weather is Great |
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Cold agglutinin |
IgM acute anemia triggered by cold seen in CLL mycoplasma pneumoniae infections infectious Mono
cold weather is MMMiserable |
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Direct coombs |
anti-Ig Ab (Coombs reagent) added to patient's blood RBCs agglutinate if RBCs are coated with Ig |
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Indirect coombs |
normal RBCs added to patient's serum if serum has anti-RBC surface Ig, RBCs agglutinate when Coombs reagent added |
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MAHA |
Pathogenesis: RBCs damaged when passing through obstructed or narrowed vessel lumina.
Seein DIC, TTP/HUS, SLE and malignant HTN
Schistocytes "helmet cells" are seen on blood smear due to mechanical destruction of RBCs |
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Macroangiopathic anemia |
Prosthetic heart valves and aortic stenosis schistocytes on peripheral blood smear |
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Neutropenia |
<1500 sepsis/postinfection, drugs, aplastic anemia, SLE, radiation |
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lymphopenia |
<1500 <3000 in children
HIV, DiGeorge, SCID, SLE, corticosteroids, radiation, sepsis, postoperative |
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Eosinopenia |
Cushing syndrome, corticosteroids |
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Acute intermittent porphyria |
sx: 5 ps painful abdomnen port wine colored urine polyneuropathy psychological disturbances precipitated by drugs, alcohol, starvation |
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Hemophilia A |
factor VIII deficiency increased PTT XLR
rx: desmopressin, factor VIII concentrate |
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hemophilia sx |
macrohemorrhage hemarhtroses (bleeding into joints) easy bruising bleeding after trauma/surgery (dental procedures) |
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Hemophilia B |
Factor IX deficiency increased PTT XLR rx: desmopressin, factor IX |
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Hemophilia C |
FActor XI deficiency AR rx: desmopressin, factor XI concentrate |
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Platelet disorders sx |
increased bleeding time microhemorrhage: mucous membrane bleeding, epistaxis, petechiae, purpura, possibly decreased platelet count |
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Bernard Soulier syndrome |
Defect in platelet plug formation decreased GpIB: defect in platelet ot vWF adhesion
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Glanzmann thrmoasthenia |
Defect in platelet plug formation decreased GpIIb/IIIa defect in platelet to platelet aggregation
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immune thrombocytopenia |
antiGpIIb/IIIa Ab-- splenic macrophage consumption of platelet-antibody complex
rx: steroids, IViG |
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TTP |
Inhibition or deficency of ADAMSTS13 ( vWF metalloprotease) decrease degradatino of vWF multimers
increase large vWF multimers--> increased platelet adhesion--> increased platelet aggregation and thrombosi
labs: schistocytes, increased LDH
sx: pentad of neurologic and renal sx, fever, thombocytopenia, and MAHA
rx: plasmapheresis, steroids |
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Von willebrand disease |
intrinsic pathway coagulation defect: decrease vWF--> increase PTT
defect in platelet plug formation
AD
mild but most common bleeding disorder
rx: desmopressin, which releases vWF stored in endothelium |
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DIC |
increased PT, PTT
widespread activation of clotting-->deficiency in clotting factors-->bleeding state
Causes: Sepsis (gram negative), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion STOP Making New Thrombi
Labs: schistocytes, increased fibrin split products (d dimers), decreased fibrinogen, decreased factors V and VII |
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antithrombin deficiency |
inherited deficiency of anththrombin no effect on PT/PTT diminishes increase in PTT following heparin administration
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Factor V Leiden |
production of mutant factor V that is resistant to degradation by activated protein C. Most common cause of hypercoagulability in whites |
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Protein C or Protein S deficiency |
Decreased ability to activate factor Va and VIIIa increased risk fo thrombotic skin necrosis with hemorrhage following administration of warfarn
skin/sub Q necrosis after warfarin administration |
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Prothrombin gene mutation |
mutation in 3' UTR increased production of prothrombin increased plasma levels and venous clots |
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Hodgkin lymphoma |
localized, single group of nodes; extranodal rare continguous spread prognosis better than non-Hodgkin lymphoma
Reed sternberg cells
Bimodal distribution (young adult and >55 y/o)
strong association with EBV
constitutional "B" signs and sx: low-grade fever, night sweats, weight loss |
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Non-Hodgkin lymphoma |
Multiple, peripheral nodes; extranodal involvement common; noncontiguous spread
majority involve B cells
peak incidence for certain subtypes at 20-40 y/o
may be associated with HIV and AI diseases
Fewer constitutional signs/sx |
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Reed Sternberg Cells |
Tumor giant cell seen in Hodgkin lymphoma
owl eyes cell
CD15, CD30 positive |
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Burkitt lymphoma |
mature B cell neoplasm adolescents/ young adults t(8,14) translocation c myc (8), heavy chain Ig (14)
Starry sky appearance, sheets of lymphocytes with interspersed macrophages
Associated with EBV
jaw lesion in endemic form in Africa; pelvis or abdomen in sporadic form |
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Diffuse large B cell lymphoma |
usually older adults, but 20% children Most common type of NH-lymphoma in adults |
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Follicular lymphoma |
Adluts t(14,18) translocation 14-heavy chain Ig, 18-BCL-2 Indolent course; BCL-2 inhibits apoptosis
presents with painless "waxing and waning" LAD nodular, small cells, cleaved nuclei |
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Mantle cell lymphoma |
Older males t(11,14) cyclin D1 (11), Ig heavy chain (14)
CD5+ |
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Adult T cell lymphoma |
adults caused by HTLV (IV drug abuse)
cutaneous lesions Japan, West Africa, Caribbean
lytic bone lesions, hypercalcemia |
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Mycosis fungoides/Sezary syndrome |
adults skin patches/plaques atypical CD4+ cells with cerebriform nuclei |
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multiple myeloma |
monoclonal plasma cell "fried egg" appearnce produces IgG or IgA most common primary tumor in those 40-50 y/o
infection primary amyloidosis (AL) punched-out lytic bone lesion son x ray M spike on SPEP Ig light chains in urine Rouleaux formation (RBCs stacked on blood smear)
"clock-face" plasma cells
CRAB
hypercalcemia Renal involvement Anemia Bone lytic lesions, Back pain MM: Monoclonal M protein |
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Waldenstrom macroglobulinemia |
M spike=IgM hyperviscosity syndrome blurred vision, Raynaud
no CRAB sx |
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MGUS
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monoclonal expansion of plasma cells, asx. may lead to MM.
no CRAB findings
develop MM at rate of 1-2% per year |
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Pseudo-Pelger-Huet anomaly |
PMNs with bilobed nuclei. seem after chemo |
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ALL: acute lymphoblastic leukemia/lymphoma |
Age: ,15y/o T cell ALL can present as mediastinal mass Associated with Down Syndrome Peripheral blood and bone marrow: increased lymphoblasts TdT+ (marker of pre-T and pre-B cells), CD10+ (pre-B only) most responsive to rx may spread to CNS and testes t(12,21)--> better prognosis |
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SLL CLL |
Age: >60 y/o most common adult leukemia CD20+, CD5+ B cell neoplasms often asx, progress slowly smudge cells in peripheral blood smear AIHA SLL same as CLL except CLL has increased peripheral blood lymphocytosis or bone marrow involvement |
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hairy cell leukemia |
Age: adults mature B cell tumor in elderly filamentous, hair-like projections causes marrow fibrosis-- dry tap on aspiration stains TRAP+ r: cladribine, pentostatin |
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AML (acute myelogenous leukemia) |
Age: 65 y/o average Auer rods peroxidase positive cytoplasmic inclusions increased circulating myeloblasts on peripheral smear risk factors: exposure to alkylating chemo, radiation, myeloproliferative disorders Down syndrome t(15,17)--> M3 AML subtype responds to alltrans retinoic acid inducing differentiation of myeloblasts DIC is common presentation |
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chronic myelogenous leukemia (CML) |
age: 45-85 y/o Philadelphia chromosome (9,22) BCR-ABL increased PMNs, metamyelocytes, basophils, splenomegaly, may accelerate and transform into AML or ALL ("blast crisis")
very low LAP Responds to imatinib (small-molecule inhibitor of bcr-abl tyrosine kinase) |
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t(8,14) |
Burkitt lymphoma (c-myc activation) |
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t(9,22) |
Philadelphia chromsome CML BCR-ABL
Philadelpha CreaML cheese |
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t(11,14) |
mantle cell lymphoma (cyclin D1 activation) |
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t(14,18) |
Follicular lymphoma (BCL-2 activation) |
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t(15,17) |
M3 type of AML responds to ATRA |
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Langerhans cell histiocytosis |
proliferative disorders of dendritic (Langerhans) cells. lytic bone lesions in child
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Chronic myeloproliferative disorders |
JAK2 mutation |
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polycythemia vera |
increased hematocrit JAK2 mutation intense itching after hot shower erythromelalgia (severe, burning pain and red-blue coloration) due to episodic blood clots in vessels of extremities
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essential thrombocytosis |
specific for overprodution of abnormal platelets bleeding, thrombosis bone marrow contains enlarged megakaryocytes |
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myelofibrosis |
Obliteration of bone marrow due to increased fibroblast activity in response to proliferation of monoclonal cell lines "teardrop" RBCs and immature forms of myeloid line dry tap massive splenomegaly |
