Glossary of USMLE - Heme Onc

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source of energy for erythrocytes?
glucose (90% anaerobically degraded to lactate; 10% by HMP shunt)
densely basophilic granules containing heparin, histamine & other vasoactive amines, and leukotrienes (LTD-4)
where are mast cells found?
cells that mediate allergic reaction?
mast cells and basophils
what does cromolyn sodium prevent?
mast cell degranulation
cells that defend against helminthic and protozoan infections?
causes of eosinophilia?
major - homozygote - beta chain is absent
when are hypersegmented neutrophils seen?
vitamin B12 deficiecncy
large, spherical, azurophilic primary granules that contain hydrolytic enzymes, lysozyme, myeloperoxidase, and lactoferrin
kidney-shaped nucleus; extensive 'frosted glass' cytoplasm; differentiates into macrophages in tissues
off-center nucleus, clock-face chromatin distribution, abundant RER and well-developed Golgi
plasma cell
multiple myeloma is what type of neoplasm?
plasma cell
what are the main inducers of the primary antibody response?
dendritic cells
what is the role of tPA?
generates plasmin, which cleaves fibrin
what does PTT measure?
intrinsic pathway
what does PT measure?
extrinsic pathway
what initiates the intrinsic pathway?
factor XII
what initiates the extrinsic pathway?
factor VII
what do proteins C and S inactivate?
Va and VIIIa; dependent on vitamin K
waht does antithrombin III inactivate?
thrombin, IXa, Xa, XIa; activated by heparin
what usually has more prognostic value - stage or grade?
degree of cellular differentiation based on histologic appearance of tumor?
degree of localization/spread based on site and size of primary lesion, spread to regional lymph nodes, presence of metastases, etc.
stage (S-spread)
macro-ovalocytes are seen in what?
megaloblastic anemia (also hypersegmented PMNs), marrow failure
helmet cells and schistocytes are seen when?
DIC, traumatic hemolysis
teardrop cells are seen when?
myeloid metaplasia with myelofibrosis
acanthocytes are seen when
spiny appearance in abetalipoproteinemia
when are target cells seen?
HALT: HbC disease, asplenia, liver disease, thalassemia
what are poikilocytes & when are they seen?
nonuniform shapes in TTP/HUS, microvascular damge, DIC
when are Burr cells seen?
etiologies of microcytic, hypochromic anemia?
iron deficiency, thalassemias, lead poisoning, sideroblastic anemias
etiologies of macrocytic anemia?
megaloblastic - vit B12/folate deficiency: drugs that block DNA synthesis (sufla,AZT), marked reticulocytosis
causes of normocytic, normochromic anemia?
enzyme defects (G6PD, PK), RBC membrane defects (e.g. hereditary spherocytosis), bone marrow disorders (aplastic anemia, leukemia), hemoglobinopathies (sickle cell), anemia of chronic disease, AI hemolytic anemia
what do decreased serum haptoglobin and increased serum LDH indicate?
RBC hemolysis
causes of aplastic anemia?
radiation, benzene, chloramphenicol, alkylating agents, antimetabolites, viral agents (parvovirus B19, EBV, HIV), Fanconi's anemia, idiopathic; may follow acute hepatitis
symptoms of aplastic anemia?
fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection
pancytopenia with normal cell morphology, hypocellular bone marrow with fatty infiltration
aplastic anemia
what is the HbS mutation?
single AA replacement in beta chain - substitution of normal glutamic acid with valine
therapies for sickle cell anemia?
hydroxyurea (increases HbF), bone marrow transplant
what percent of blacks carry the Hb trait? what percent have the disease?
8%, 0.2%
crew cut on skull x-ray?
sickle cell anemia, thalassemias - due to marrow expansion from increased erythropoeisis
what is the defect in beta thalassemia minor? major?
minor - heterozygote - beta chain is underproduced
why does cardiac failure occur in thalassemia?
secondary hemochromatosis
marked erythrocytosis, moderate increase in granulocytes and platelets, splenomegaly, decreased epo?
polycythemia vera
labs in anemia of chronic disease?
decreased TIBC, increased ferritin, low serum iron
findings in hemolytic anemia?
increased serum bilirubin, increased reticulocytes (marrow compensating for anemia)
test for AI hemolytic anemia?
Coombs positive
chronic anemia seen in SLE, CLL, or with certain drugs (e.g. alpha methyldopa)
warm agglutinin (IgG)
anti-Ig Ab added to patient's RBCs agglutinate if RBCs are coated with IgG
direct Coombs' test
normal RBCs added to patient's serum agglutinate if serum has anti-RBC surface IgG
indirect Coombs' test
microvascular hemolysis seen in DIC, TTP/HUS, SLE, or malignant hypertension
microangiopathic anemia
what is seen on blood smear in microangiopathic anemia?
schistocytes (helmet cells)
what is increased in paroxysmal nocturnal hemoglobinuria?
urine hemosiderin
activation of coagulation cascade leading to microthrombi and global consumption of platelets, fibrin, and coagulation factors
lab findings in DIC?
increased PT, PTT, fibrin split products (D dimers); decreased platelet count
what is seen on blood smear in DIC?
helmet-shaped cells and schistocytes
antiplatelet antibodies, megakaryocytes?
schistocytes, increased LDH, neuro sx
microhemorrhage - mucous membrane bleeding, epistaxis, petechiae, purpura, increased bleeding time
platelet abnormalities - ITP, TTP, DIC, aplastic anemia, drugs (e.g. immunosuppressive agents)
factor VIII deficiency
hemophilia A
factor IX deficiency
hemophilia B
von Willebrand's disease
mild; most common bleeding disorder; deficiency of vWF - defect of platelet adhesion and decreased factor VIII survival
macrohemorrhage - hemarthroses (bleeding into joints), easy bruising, increased PT, PTT
coagulation factor deficiencies
Bernard-Soulier disease?
defect of platelet adhesion (decreased GP Ib) - prolonged bleeding time
Glanzmann's thrombasthenia
defect of platelet aGgregation - decreased GP IIb-IIIa - prolonged bleeding time
decresed platelet count, increased bleeding time?
lab findings in hemophilia A or B?
normal platelet count, bleeding time, PT, increased PTT
increased bleeding time and increased PTT
von Willebrand's disease
findings in DIC
decreased platelet count, increased bleeding time, PT, and PTT
PT measures what?
extrinsic pathway - factors, II, V, VII, X
PTT measures what?
intrinsic pathway
origin/markers of RS cells?
B cell origin - CD30+, CD15+
good prognosis in Hodgkins?
increased lymphocytes, decreased RS cells
50% of cases of Hodgkin's are associated with what?
what is non-Hodgkin's associated with?
HIV and immunosuppression
peak incidence of non-Hodgkin's?
20-40 years old
peak incidence of Hodgkin's?
bimodal distribution - young and old
most common type of Hodgkin's?
nodular sclerosing type
collagen banding, lacunar cells in Hodgkin's?
nodular sclerosing type - most common in young women
numerous RS cells are found in what type of Hodgkin's?
mixed cellularity (25% of cases)
Hodgkin's in older males with disseminated disease; poor prognosis?
lymphocyte depleted
cell type in SLL, follicular lymphoma (small cleaved cell)?
B cells
this is like CLL with focal mass, low grade
small lymphocytic lymphoma
most common in children; commonly presents with ALL and mediastinal mass; very aggressive T cell lymphoma
lymphoblastic lymphoma
cell type in diffuse large cell lymphoma? who does it affect
80% B cells; 20% T cells; usually older adults, but 20% occur in children
starry sky appearance - sheets of lymphocytes with interspersed macrophages?
Burkitt's lymphoma
jaw lesion in endemic from in Africa; pelvis or abdomen in sporadic form
Burkitt's lymphoma
cell type in Burkitt's lymphoma?
B cells - most often affects kids
leukemia most responsive to therapy?
children, lymphoblasts; may spread to CNS and testes
auer rods, myeloblasts, adults
leukemia affecting older adults; lymphadenopathy, hepatosplenomegaly, few symptoms, indolent course?
findings on blood smear in CLL?
smudge cells
leukemia that can cause warm antibody AI hemolytic anemia?
leukemia that presents with increased neutrophils and metamelocytes, splenomegaly
what is low in CML
leukocytic alkaline phosphatase
peroxidase-positive cytoplasmic inclusions in granulocytes adn myeloblasts
Auer rods
in which type of AML are Auer rods primarily seen?
acute promyelocytic leukemia (M3)
treatment of this leukemia can release Auer rods, causing DIC
monoclonal plasma cell (fried egg appearance) cancer that arises in the marrow and produces large amounts of IgG (55%) or IgA (25%)
multiple myeloma
most common primary tumor arising from bone in adults
multiple myeloma
these are laminated, concentric, calcific spherules
psammoma bodies
where are psammoma bodies seen?
PSaMMoma: papillary (thyroid), serous (ovary), meningioma, mesothelioma

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