Term
| Universal recipient blood group |
|
Definition
| AB (no antibodies in plasma) |
|
|
Term
| Universal donor blood group |
|
Definition
|
|
Term
| Cause of hemolytic disease in the newborn |
|
Definition
| Anti-Rh IgG made by mom, cross placenta |
|
|
Term
| Response to Incompatible blood transfusion |
|
Definition
Immunologic response
hemolysis
renal failure
shock
death |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| hereditary elliptocytosis |
|
|
Term
| RBC form: macro-ovalocyte |
|
Definition
| Megaloblastic anemia, marrow failure |
|
|
Term
|
Definition
aka schistocytes
TTP/HUS, DIC, traumatic hemolysis |
|
|
Term
|
Definition
G6PD deficiency, a-Thalassemia
also Heinz bodies (denatured HB precipitation) |
|
|
Term
|
Definition
| Myeloid metaplasia with myelofibrosis |
|
|
Term
|
Definition
aka spur cell
Spiny appearance in liver disease and abetalipoproteinemia |
|
|
Term
|
Definition
HB C disease
Asplenia
Liver disease
Thalassemia |
|
|
Term
|
Definition
|
|
Term
| RBC form: Basophilic stippling |
|
Definition
| Thalassemias, ACD, Iron deficiency, Lead poisoning |
|
|
Term
| Denatured HB precipitates? What causes this? |
|
Definition
Heinz bodies
oxidation of Fe from ferrous to ferric form leads to HB denaturation and RBC membrane damage
seen in G6PD deficiency (susceptible to oxidative stress) and alpha-Thalassemia |
|
|
Term
| Basophilic nuclear remnants found in RBC's? Who has these? |
|
Definition
Howell jolly bodies
seen in patients with functional hyposplenia/asplenia (ie sickle cell) |
|
|
Term
Anemia caused by iron deficiency
labs? |
|
Definition
microcytic
decreased serum Fe, increased TIBC, decreased ferritin |
|
|
Term
| Anemia caused by thalassemias |
|
Definition
|
|
Term
| Anemia caused by lead poisoning |
|
Definition
|
|
Term
| Anemia caused by sideroblastic anemias |
|
Definition
microcytic
failure to completely form heme |
|
|
Term
Anemia caused by ACD
labs? |
|
Definition
microcytic or normocytic
Increased ferritin, decreased TIBC, increased storage of iron in marrow macrophages, increased hepcidin |
|
|
Term
Anemia caused by B12/folate deficiency
association? |
|
Definition
macrocytic
associated with hypersegmented PMNs (>5 lobes)
B12 - neuro problems, glossitis, increased MMA and homocysteine |
|
|
Term
Anemia caused by sulfa drugs/phenytoin/AZT
Why? |
|
Definition
macrocytic
interfere with DNA synthesis |
|
|
Term
| Anemia caused by acute hemorrhage |
|
Definition
|
|
Term
| Anemia caused by G6PD deficiency |
|
Definition
normocytic
X linked enzyme defect |
|
|
Term
| Anemia caused by PK deficiency |
|
Definition
normocytic
AR enzyme defect |
|
|
Term
Anemia caused by RBC membrane defects
example? |
|
Definition
normocytic
hereditary spherocytosis |
|
|
Term
Anemia caused by bone marrow disorders
example? |
|
Definition
normocytic
aplastic anemia, leukemia |
|
|
Term
Anemia caused by hemoglobinopathies
Example? |
|
Definition
normocytic
sickle cell disease |
|
|
Term
Anemia caused by autoimmune hemolytic anemia
labs? |
|
Definition
normocytic
decreased serum haptoglobin
increased serum LDH
positive direct coombs |
|
|
Term
| Labs seen in RBC hemolysis |
|
Definition
decreased serum haptoglobin (binds free HB and is cleared)
increased serum LDH (intracellular enzyme released with hemolysis)
Use direct coombs test to distinguish between immune vs. nonimmune |
|
|
Term
| Binds to free HB and is cleared |
|
Definition
|
|
Term
| Binds ferroportin and prevents Fe export from intracellular stores |
|
Definition
|
|
Term
| inhibits ferrochelatase and ALA dehydrase |
|
Definition
Lead poisoning
leads to microcytic anemia |
|
|
Term
| Conditions in which specific enzymes needed in heme synthesis are defective leading to accumulation of proximal intermediates |
|
Definition
|
|
Term
| Describe the progression of IDA |
|
Definition
Decreased ferritin
decreased serum Fe, increased TIBC
microcytosis, hypochromia, increased RDW
ANEMIA
correction with Fe replacement occurs in the reverse order |
|
|
Term
| Presentation: atrophic glossitis, PICA, koilonychia |
|
Definition
IDA
PICA - ice chewing
Kolionychia - spoon shaped nails |
|
|
Term
| Accumulated substrate in urine: Coprophorphyrin and ALA |
|
Definition
Lead poisoning
Affected enzyme: Ferrochelatase and ALA dehydratase |
|
|
Term
| Accumulated substrate in urine: prophogilinogen and delta-ALA |
|
Definition
Acute intermittent porphyria
Affected enzyme: uroporphyrinogen I synthase
Symptoms: Painful abdomen, Pink urine, polyneuropathy, psych disturbances, precipitated by drugs |
|
|
Term
| Accumulated substance in urine: Uropophyrin (tea colored) |
|
Definition
Porphyria cutanea tarda
Affected enzyme: uroporphyrinogen decarboxylase |
|
|
Term
| Increased delta-aminolevulinic acid causes what? |
|
Definition
increased GABA and increased oxidation of membrane lipids
leads to neuronal damage
seen in Lead poisoning and AIP |
|
|
Term
Decreased serum iron,
increased TIBC/transferrin,
Decreased ferritin,
decreased % transferrin saturation |
|
Definition
|
|
Term
Decreased serum iron,
Decreased TIBC/transferrin,
Increased ferritin,
Normal % transferrin saturation |
|
Definition
Anemia of chronic disease
body locks up iron to prevent pathogen from acquiring circulating iron |
|
|
Term
| Absence of staining with Prussian blue of bone marrow |
|
Definition
|
|
Term
Increased serum iron,
Decreased TIBC/transferrin,
Increased ferritin,
increased % transferrin saturation |
|
Definition
|
|
Term
normal serum iron,
increased TIBC/transferrin,
normal ferritin,
decreased % transferrin saturation |
|
Definition
|
|
Term
| Causes of aplastic anemia |
|
Definition
radiation, benzene,
chloramphenicol, alkylating agents,
antimetabolites, viral agents (parvo, EBV, HIV), following acute hepatitis
Fanconi's anemia (inherited defect in DNA repair),
idiopathic (immune mediated, primary SC defect), |
|
|
Term
| Pancytopenia with normal cell morphology, hypocellular bone marrow with fatty infiltration |
|
Definition
|
|
Term
| Treatment for aplastic anemia |
|
Definition
1. withdraw offending agent
2. immunosuppressive regimens (antithymocyte gloulin, cyclosporine)
3. allogenic BM transplant
4. RBC, plt transfusion
5. G-CSF or GM-CSF |
|
|
Term
| Genetic cause in sickle cell anemia |
|
Definition
| normal glutamic acid with valine in the B chain of HB |
|
|
Term
|
Definition
| low oxygen or dehydration |
|
|
Term
| Advantage of being heterozygous sickle cell trait |
|
Definition
|
|
Term
| Complications of sickle cell anemia |
|
Definition
1. aplastic crisis (parvo B19)
2. autosplenectomy and increased risk of encapsulated organism infection
3. Salmonella osteomyelitis
4. painful crisis (vasoocculsive)
5. renal papillary necrosis
6. splenic sequestration crisis |
|
|
Term
| Sickle cell anemia therapies |
|
Definition
1. Hydroxyurea (to increase HbF)
2. BM transplant |
|
|
Term
| What % or African americans carry HBS trait? disease? |
|
Definition
|
|
Term
|
Definition
due to marrow expansion from increased erythropoiesis
seen in Sickle cell anemia and thalassemias |
|
|
Term
| Newborn presentation of sickle cell anemia |
|
Definition
Asymptomatic - increased HbF, decreased HbS
present around 6 months old |
|
|
Term
| Type of hemolysis seen in sickle cell anemia |
|
Definition
Intravascular and extravascular
Sickled cells have shortened lifespan (20d) |
|
|
Term
| Disease presentation of HbC, HbSC compared to HbSS? |
|
Definition
milder
HbC due to different B chain mutation |
|
|
Term
| Genetic defect in a-thalassemia |
|
Definition
| Large deletion of a-globin gene (4 genes total) |
|
|
Term
|
Definition
| B4-tetramer, lacks 3 a-globin genes |
|
|
Term
|
Definition
y4-tetramers, lacks all 4a globin genes
results in hydrops fetalis and intrauterine fetal death |
|
|
Term
|
Definition
deletion of all 4a genes, y4-tetramer Hb
intrauterine fetal death |
|
|
Term
| Blood dyscrasia prevalent in Asia and Africa |
|
Definition
|
|
Term
| Blood dyscrasia prevalent in Mediterranean populations? |
|
Definition
|
|
Term
| Genetic defect in B-thalassemia? |
|
Definition
| Mutation in splicing sites and promoter sequences |
|
|
Term
| B-thal minor v. B-thal major? |
|
Definition
Minor - B chain underproduced
Major - B chain absent
both compensate with increased HbF (a2y2), but this is inadequate |
|
|
Term
| Clinical outcome of B-thal major? |
|
Definition
severe anemia, requires transfusions
cardiac failure due to secondary hemochromatosis
marrow expansion (crew cut x ray) leads to skeletal deformities |
|
|
Term
| Distribution of Hb types in normal adult |
|
Definition
HB A - a2B2 (97%)
HB A2 - a2d2 (2.5%)
Hb F - a2y2 (<1%)
HbF predominates until 6 months of age |
|
|
Term
| Labs seen in Intravscular hemolysis |
|
Definition
greatly increased indirect bili, LDH, urine hemosdirin and HB
greatly decreased haptoglobin |
|
|
Term
| Labs seen in extravascular hemolysis? |
|
Definition
moderately increased indirect bili, LDH
mildly decreased/normal haptoglobin
normal urine hemosiderin/HB |
|
|
Term
| Autoimmune hemolytic anemia caused by IgG |
|
Definition
Warm agglutinin
seen in SLE, CLL, or certain drugs (a-methyldopa)
mostly extravascular
accelerated RBC destruction in liver Kupffer cells and spleen |
|
|
Term
| Autoimmune hemolytic anemia seen with IgM |
|
Definition
cold agglutinin
seen with Mycoplasma pneumoniae or Mono
Complement involved |
|
|
Term
| anti-Ig Ab added to patients RBC's causes agglutination |
|
Definition
+Direct coombs test
seen in AIHA |
|
|
Term
| normal RBCs agglutinate when patient's serum is added |
|
Definition
+ indirect Coombs test
seen in AIHA |
|
|
Term
| Erythoroblastosis fetalis |
|
Definition
seen in newborn
due to Rh or other blood antigen incompatibility
mothers Ab attack fetal RBCs |
|
|
Term
Defect in ankyrin, band 3.1, or spectrin
Inheritance? disease? clinical consequence? |
|
Definition
Hereditary spherocytosis
AD
Intrinsic, extravascular hemolysis
RBC's small, no central pallor (less membrane), increased MCHC and RDW
associated with splenomegaly, aplastic crisis, Howell jolly body following splenectomy |
|
|
Term
| Increased MCHC and RDW, associated with splenomegaly and aplastic crisis. Coombs negative. |
|
Definition
Hereditary spherocytosis (AD)
confirm with osmotic fragility test |
|
|
Term
| Diagnosis confirmed with osmotic fragility test |
|
Definition
Hereditary spherocytosis (AD)
coombs negative |
|
|
Term
| Family history of premature pigment gallstones along with microcytic anemia, increased MCHC and RDW |
|
Definition
| Hereditary spherocytosis (AD) |
|
|
Term
Increased sensitivity of RBCs due to lytic activity of complement
Disease? Cause? Clinical consequence? |
|
Definition
Paroxsysmal nocturnal hemoglobinuria
-impaired synthesis of GPI anchor/decay accelerating factor in RBC membrane
-leads to intravascular hemolysis (hypoventilation at night causes decreased pH and less stable RBC membrane)
red urine |
|
|
Term
| Impaired synthesis of GPI anchor/DAF |
|
Definition
Paroxysmal nocturnal hemoglobinuria
increased sensitivity of RBCs to lytic activity of complement |
|
|
Term
| Name the microangiopathic anemias |
|
Definition
DIC, TTP/HUS
SLE, malignant hypertension
intravascular hemolysis, schisotcytes (mechanical damage of RBCs passing through obstructed/narrowed vessel lumina) |
|
|
Term
|
Definition
activation of coagulation cascade
microthrombi
global consumption of platelets, fibrin, coag factors |
|
|
Term
|
Definition
Sepsis (G-)
Trauma
Obstetric complication
Pancreatitis (acute)
Malignancy
Nephrotic syndrome
Transfusion
STOP Making New Thrombi |
|
|
Term
|
Definition
Increased PT, PTT and fibrin split product (D-Dimer)
decreased platelet count
helmet shaped cells, schistocytes |
|
|
Term
| Large platelets with thrombocytopenia, nl PT and aPTT |
|
Definition
| ITP (peripheral platelet destruction, IgG anti-GpIIP/IIIa Ab, increased megakaryocytes |
|
|
Term
| Microhemorrhage: mucous membrane bleeding, epistaxis, petechiae, purpura, increased bleeding time |
|
Definition
| Platelet abnormalities (ITP, TTP, DIC, aplastic anemia, Drugs - immunosuppressives) |
|
|
Term
Deficiency of vWF cleaving metalloproteinase
disease? pathophys? clinical? |
|
Definition
TTP (ADAMTS 13= enzyme)
Enzyme def. leads to excess of large vWF multimers, increased platelet aggregation, thrombosis, and schistocyte formation, increased LDH
neuro, renal and fever |
|
|
Term
|
Definition
Thrombocytopenia
Fever
altered mental status
renal insufficiency
thrombi |
|
|
Term
| Hemarthroses, easy bruising, increased PT/aPTT |
|
Definition
| Coagulation defect (Hem A, Hem B, vWD) |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| Most common bleeding disorder |
|
Definition
vWD (mild)
deficiency of vWF --> defect of platelet adhesion and decreased VIII survival |
|
|
Term
| Plt count, BT, PT, and PTT: thrombocytopenia |
|
Definition
Plt - low
BT - increased
PT - normal
PTT - normal |
|
|
Term
| Plt count, BT, PT, and PTT: Hem A or B |
|
Definition
Plt - normal
BT - normal
PT - normal
PTT - increased |
|
|
Term
| Plt count, BT, PT, and PTT: vWD |
|
Definition
Plt - normal
BT - increased
PT - normal
PTT - normal/increased (carries F VIII) |
|
|
Term
| Plt count, BT, PT, and PTT: DIC |
|
Definition
Plt - low
BT - increased
PT - increased
PTT - increased |
|
|
Term
| Plt count, BT, PT, and PTT: vit K deficiency |
|
Definition
Plt - normal
BT - normal
PT - increased
PTT - increased |
|
|
Term
| Plt count, BT, PT, and PTT: Berndard Soulier disease |
|
Definition
Plt - decreased
BT - increased
PT - normal
PTT - normal
defect in GP1b |
|
|
Term
| Plt count, BT, PT, and PTT: Glanzmann's thromboasthenia |
|
Definition
Plt - normal
BT - increased
PT - normal
PTT - normal
defect in GPIIb/IIIa |
|
|
Term
|
Definition
F I, II, V, VII, X
extrinsic |
|
|
Term
|
Definition
F VII, XIII
intrinsic (includes all others) |
|
|
Term
|
Definition
Glanzmann's thromboasthenia
defect in platelet-to-platelet aggregation |
|
|
Term
|
Definition
Bernard soulier disease
defect in platelet-to-collagen adhesion |
|
|
Term
|
Definition
vWF
defect in platelet to collagen adhesion |
|
|
Term
|
Definition
Ristoceten selectively activated Gp1b
in BS - no platelet activation
in GT - only ristoceten stimulates platelets
in vWD - decreased action |
|
|
Term
| Vit K deficiency affects which factors? |
|
Definition
II, VII, IX, X, protein C and S
increases PT and PTT |
|
|
Term
|
Definition
Hodgkin's disease
distinctive tumor giant cell; binucleate or bilobed with 2 halves as mirror images, survival independent of antigen R signaling, release cytokines attracting reactive mixed inflammatory response
CD30+ and CD15+ B cell origin
variants = lacunar cells in nodular sclerosis |
|
|
Term
| Cells necessary to diagnose Hodgkin's disease |
|
Definition
|
|
Term
| Markers and genesis of Reed sternberg cells? |
|
Definition
CD30+ and CD15+, B cell origin
survival independent of antigen R signaling
release cytokines attracting reactive mixed inflammatory response |
|
|
Term
| Hodgkins or Non-Hodgkins: presence of reed sternberg cells |
|
Definition
|
|
Term
| Hodgkins or Non-Hodgkins: localized, single group of nodes, extranodal rare |
|
Definition
|
|
Term
| Hodgkins or Non-Hodgkins: may be associated with HIV and immunosuppression |
|
Definition
|
|
Term
| Hodgkins or Non-Hodgkins: extranodal involvement common, noncontiguous spread |
|
Definition
|
|
Term
| Hodgkins or Non-Hodgkins: B symptoms common |
|
Definition
|
|
Term
| Hodgkins or Non-Hodgkins: B symptoms less common |
|
Definition
|
|
Term
| Hodgkins or Non-Hodgkins: mediastinal lymphadenopathy |
|
Definition
|
|
Term
| Cellular signs of good prognosis in HL? |
|
Definition
Increased lymphocytes
decreased RS cells |
|
|
Term
|
Definition
| Nodular sclerosing (collagen banding, lacunar cells, F>M, young adults) |
|
|
Term
|
Definition
Nodular sclerosing
most common type of HL |
|
|
Term
| Prognosis of Nodular sclerosing HL |
|
Definition
|
|
Term
| Predominance of RS and lymphocytes in nodular sclerosing HL? |
|
Definition
|
|
Term
| Lacunar cells are seen in which HL? |
|
Definition
|
|
Term
| Characteristics of mixed cellularity HL |
|
Definition
| RS ++++, lymphocytes +++, intermediate prognosis, EBV related, elderly |
|
|
Term
|
Definition
Mixed cellularity (25%)
most common is nodular sclerosing (65-75%) |
|
|
Term
| Characteristics of lymphocyte depleted HL? |
|
Definition
| RS cells high compared to lymphocytes, poor prognosis, older males with disseminated disease, EBV and HIV+ |
|
|
Term
|
Definition
Lymphocyte depleted
see spindled and bizarre cells |
|
|
Term
| Characteristics of lymphocyte predominant |
|
Definition
| RS +, lymphocyte ++++, excellent prognosis, <35 yo males |
|
|
Term
| How does EBV play a role in HL? |
|
Definition
| upregulates NF-kB, leads to lymphocyte survival |
|
|
Term
|
Definition
|
|
Term
|
Definition
| bimodal: young (NS, LPHL) and old (>70, MCHL, LDHL, LPHL) |
|
|
Term
| Why is lymphocyte predominant HL non classic? |
|
Definition
| germinal center B cells without deleterious somatic mutations, maintain Ig expression, RS survival depends on antigen selection process |
|
|
Term
| Lymphocyte and histocyte cell |
|
Definition
See in LPHL
aka popcorn cell |
|
|
Term
| CD markers in classic HL? nonclassic? |
|
Definition
Classic: CD15+, CD30+
Nonclassic: CD45+, CD20+ |
|
|
Term
| Treatment of Lymphocyte predominant HL? |
|
Definition
excision, limited radiation, rituximab
5% progress to DLBC lymphoma |
|
|
Term
| Most common primary tumor arising within the bone in elderly? |
|
Definition
Multiple myeloma
elderly = >40-50 |
|
|
Term
| Hypercalcemia, Renal insufficiency, Anemia, Bone pain |
|
Definition
|
|
Term
| Punched out lytic bone lesions on x-ray |
|
Definition
|
|
Term
| Monoclonal Ig spike (M protein) |
|
Definition
Multiple meyloma
55% - IgG and 25% IgA |
|
|
Term
|
Definition
|
|
Term
|
Definition
| seen in Multiple myeloma and ACD |
|
|
Term
| Waldenstrom's macroglobulinemia |
|
Definition
M spike = IgM (which leads to hyperviscosity symptoms)
no lytic bone lesions
hypervisous: chronic nasal/gum bleeding, dizzy, HA, ataxia, diplopia, can lead to HF |
|
|
Term
| M spike present but asymptomatic |
|
Definition
Monoclonal gammopathy of undetermined significance (MGUS)
no treatment needed
1%/ year progress to MM |
|
|
Term
| Multiple myeloma is associated with what? |
|
Definition
Primary amyloidosis
light chain variable region, monoclonal, gamma |
|
|
Term
| Clinical consequences of primary amyloidosis |
|
Definition
associated with MM
leads to HF, hepatosplenomegaly, macroglossia, purpura, nephrotic syndrome, peripheral neuropathy
EKG: low voltage, anteroseptal force loss |
|
|
Term
Small lymphocytic lymphoma
Class? Population? Cell type? |
|
Definition
|
|
Term
| Occupational hazard of working as a farmer, with rubber, or benzene |
|
Definition
Small Lymphocytic Lymphoma (NHL)
no radiation though |
|
|
Term
Class? Population? Cell type?
Follicular lymphoma |
|
Definition
NHL with small cleaved cells
Adults (60 M)
B cells
t(14:18), bcl-2 expression (which inhibits apoptosis)
25% progress to DLBCL |
|
|
Term
|
Definition
Follicular lymphoma (small cleaved cells)
bcl-2 expression which inhibits apoptosis |
|
|
Term
Class? Population? Cell type?
Diffuse Large cell lymphoma |
|
Definition
usually older sdults (20% in kids though)
80%B cells, 20% T cells (mature)
aggressive (but many are curable)
DLBCL is most common NHL |
|
|
Term
|
Definition
|
|
Term
Class? Population? Cell type?
Mantle cell lymphoma |
|
Definition
NHL
Adults (60M)
B cells
t(11:14) cyclin D1
poor prognosis (incurable and aggressive), CD5+ |
|
|
Term
| Incurable and aggressive lymphoma |
|
Definition
| Mantle Cell lymphoma (NHL) |
|
|
Term
|
Definition
Mantle cell lymphoma (incurable and aggressive)
cyclin D1 |
|
|
Term
Class? Population? Cell type?
lymphoblastic lymphoma |
|
Definition
NHL
most often kids
T cells (immature)
commonly presents with ALL and mediastinal mass
very aggressive (70% survival) |
|
|
Term
Class? Population? Cell type?
Burkitt's lymphoma |
|
Definition
NHL
Most often kids
B cells
t(8:14) c myc next to heavy chain Ig gene(14)
starry sky appearance |
|
|
Term
|
Definition
Burkitt's lymphoma
c-myc moves next to heavy chain Ig gene (14)
Starry sky appearance |
|
|
Term
|
Definition
seen in Burkitt's lymphoma
sheets of squared lymphocytes with interspersed benign macrophages |
|
|
Term
|
Definition
Burkitt's lymphoma
jaw lesions in endemic form in Africa (5M)
pelvis or abdomen in sporadic form |
|
|
Term
| Where does sporadic form Burkitt's lymphoma present? |
|
Definition
pelvis or abdomen
most rapidly growing neoplastic cells |
|
|
Term
| Danger of rapidly dying tumor |
|
Definition
tumor lysis syndrome
often seen in Burkitt's lymphoma
leads to abnormal electorlytes and renal failure |
|
|
Term
| Most rapidly growing neoplastic cells |
|
Definition
Burkitt's lymphoma
sporadic - abdominal/pelvis masses
endemic - jaw lesions |
|
|
Term
|
Definition
philadelphia chromosome
CML (bcr-abl hybrid)
constitutively active tyrosine kinase |
|
|
Term
|
Definition
M3 type of AML (early complication is DIC)
responsive to all-trans retinoic acid)
PML and RARa, |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| Best guess for a kid with leukemia? |
|
Definition
|
|
Term
| Increased WBC count with left shift and increased leukocyte alkaline phosphatase? |
|
Definition
| Leukemoid reaction (benign, exaggerated leukocyte response, see Dohle bodies) |
|
|
Term
| Leukemia most responsive to treatment |
|
Definition
|
|
Term
| Where does ALL spread to? |
|
Definition
|
|
Term
|
Definition
B: most common, no organomegaly or mediastinal masses
T: organomegaly, mediastinal masses, poor prognosis |
|
|
Term
|
Definition
marker of pre-T and pre-B cells
seen in ALL |
|
|
Term
| Often presents with gingival infiltration? |
|
Definition
|
|
Term
|
Definition
responds to all trans retinoic acid (vit A)
often presents with DIC |
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Term
|
Definition
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Term
|
Definition
Older adults (>60)
lymphadenopathy, hepatosplenomegaly
few symptoms, generally indolent course
increased smudge cells in peripheral blood smear
most common chronic leukemia |
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Term
| Most common chronic anemia |
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Definition
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Term
| How do you distinguish pre B from pre T cells? |
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Definition
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Term
| Leukemia associated with warm Ab AIHA |
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Definition
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Term
| Presents with increased neutrophils, metamyelocytes, basophils, splenomegaly |
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Definition
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Term
| Progression and treatment of CML |
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Definition
Untreated: asyx: 3-7 yrs, accelerates: 1-2 yrs, blast crisis and death
Treated with imatinib (anti-bcr-abl)
may accelerate and transform to AML (2/3) or ALL (1/3) (blast crisis) |
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Term
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Definition
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Term
| What distinguishes AML from Leukomoid reaction |
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Definition
| CML - very low leukocyte alkaline phosphatase |
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Term
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Definition
| seen in CML (increased neutrophils, metamyelocytes, basophils) |
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Term
| Mature B cell tumor of elderly |
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Definition
Hairy cell leukemia
cells have filamentous hair like projections
stains TRAP tartrate-resistant acid phosphatase + |
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Term
| Elderly man with pancytopenia, splenomegaly, and no lymphadenopathy |
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Definition
Hairy cell leukemia
Other key words:
dry tap aspirate
fried egg appearance
red pulp infiltrate, blood lakes
TRAP+ |
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Term
| Leukemia affecting <15 y.o. |
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Definition
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Term
| Leukemia affecting 30-60 y.o. |
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Definition
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Term
| Leukemia with median onset of 60 y.o. |
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Definition
| AML (80% of adult leukemias) |
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Term
| Leukemia affecting >60 y.o. |
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Definition
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Term
| Peroxidase+ cytoplasmic inclusions in granulocytes and myeloblasts |
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Definition
Auer bodies (commonly seen in AML, especially M3 - acute promyelocytic leukemia)
treatment of M3 can release auer rods, causing DIC |
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Term
| Proliferative disorders of dendritic (Langerhans) cells from monocyte lineage |
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Definition
Langerhans cell histiocytoses
Histiocytosis X |
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Term
|
Definition
| expressed by defective cells in Langerhans cell histiocytosis/histiocytosis X |
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Term
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Definition
tennis rackets on EM
characteristic of Langerhans cell histiocytosis/X (proliferative disorder of dendritic cells from monocyte lineage) |
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Term
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Definition
older term for Histiocytosis X
birbeck granules (tennis rackets)
S-100 and CD1a |
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Term
| Hand-Schuller-Christian disease |
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Definition
older term for Histiocytosis X
birbeck granules (tennis rackets)
S-100 and CD1a |
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Term
|
Definition
older term for Histiocytosis X
birbeck granules (tennis rackets)
S-100 and CD1a |
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Term
| Increased RBCs, WBCs, and platelets with +JAK2 mutation |
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Definition
Polycythemia vera (myeloproliferative disorder)
<20% blasts
60 yo Jewish M
decreased/Nl Epo |
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Term
| Increased platelets but normal RBCs and WBCs |
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Definition
Essential Thrombocytopenia (60F)
+JAK2 mutation (V617F) |
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Term
| Decreased RBCs, variable WBCs and platelets, +JAK2 |
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Definition
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|
Term
Decreased RBCs, increased WBCs and platelets
Disease and gene? |
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Definition
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Term
|
Definition
Polycythemia vera
hyperviscosity (all cell lines increased) |
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Term
| specific proliferation of megakaryocytes |
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Definition
Essential thrombocytosis
platelets are large, hypogranular, and abnormal
clustered Megs in BM |
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Term
|
Definition
constitutively active tyrosine kinase
increased cell division and inhibition of apoptosis |
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Term
| Pseudo pelger-huet cells and cytopenias and hypolobated PMNs |
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Definition
Myelodysplastic syndrome (<20% blasts)
hypercellular BM with excessive apoptosis |
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Term
| 5q deletion in myelodysplastic syndrome |
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Definition
good prognosis
treat with lanalidomide |
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Term
| WBC differential from highest concentration to lowest |
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Definition
Neutrophils
Lymphocytes
Monocytes
Eosinophils
Basophils
neutrophils like making everything better |
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Term
| What allows for easy gas exchange in RBCs? |
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Definition
| Large surface area:volume ratio |
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Term
| Source of energy for RBCs |
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Definition
glucose
90% anaerobically degraded to lactate
10% by HMP shunt |
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Term
| What is the physiologic chloride shift? |
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Definition
Allows RBCs to transport CO2 from periphery to lungs for elimination
membrane of RBC has chloride-bicarb antiport |
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Term
| Cells that mediate allergic reaction |
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Definition
basophil
<1% of all leukocytes
mast cells |
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Term
| What type of nucleus is in the basophil? |
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Definition
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|
Term
| What is in the basophilic granules? |
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Definition
heparin
histamine (vasodilator)
vasoactive amines
leukotrienes (LTD-4) |
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|
Term
| Resemble basophils structurally and functionally but no the same cell type |
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Definition
mast cell
found in some tissue |
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Term
| What is released upon degranulation of mast cells? |
|
Definition
| histamine, heparin, eosinophil chemotactic factors |
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Term
| What cell is involved in Type I hypersensitivity? |
|
Definition
Mast cells
IgE can bind to membrane
degranulation releases histamine, heparin, and eosinophil chemotactic factors |
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Term
| What can be used to prevent degranulation of mast cells? |
|
Definition
Cromolyn sodium
used to treat asthma |
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Term
| What is the role of eosinophils? |
|
Definition
1. defend against helminthic and protozoan infections (major basic protein)
2. highly phagocytic for antigen-antibody complexes
3. produce histaminase and arylsulfatase to help limit reaction following mast cell degranulation |
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Term
|
Definition
Neoplastic
Asthma
Allergic processes
Collagen vascular diseases
Parasites |
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|
Term
|
Definition
|
|
Term
| Type of nucleus in eosinophil |
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Definition
|
|
Term
| What do the granules in neutrophils contain? |
|
Definition
large, spherical, azuophilic granules = lysosomes
contain hydrolytic enzymes, lysozyme, myeloperoxidase and lactoferrin |
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Term
|
Definition
acute inflammatory response cell
phagocytic
granules = lysosomes with hydrolytic enzymes, lysozyme, myeloperoxidase, lactoferrin |
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Term
|
Definition
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|
Term
|
Definition
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|
Term
| Has a kidney shaped nucleus and extensive frosted glass cytoplasm |
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Definition
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|
Term
|
Definition
differentiates into macrophage in tissue
has kidney shaped nucleus and frosted glass cytoplasm |
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Term
|
Definition
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Term
|
Definition
1. phagocytoses bacteria, cell debris, and senescent RBCs, damaged cells and tissues
2. Can function as APC via MHC II |
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Term
| Activated by y-interferon |
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Definition
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|
Term
| Small cytoplasmic fragment derived from megakaryocytes |
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Definition
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Term
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Definition
| 1. primary heomstasis (activated by endothelial injury, aggregates with other platelets, interacts with fibrinogen to form hemostatic plug) |
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Term
|
Definition
in platelets
contain ADP and calcium |
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Term
|
Definition
In platelets
contain vWF and fibrinogen |
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|
Term
| How much of the platelet pool is stored in the spleen? |
|
Definition
1/3
life span of platelet = 8-10d |
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Term
|
Definition
| result from thrombocytopenia or platelet dysfunction |
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Term
| Main inducers of primary antibody response |
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Definition
Dendritic cells
called langerhans cells on skin
express MHC II and Fc receptor on surface |
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Term
|
Definition
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Term
|
Definition
Arises in stem cells of bone marrow
matures in marrow
migrates to peripheral lymphoid tissue (follicles of lymph nodes, white pulp of spleen, unencapsulated lymphoid tissue)
Encounters antigen, differentiates into plasma cells
produces antibody
has memory
can function as an APC via MHC II |
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Term
| clock face chromatin and off center nucleus |
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Definition
Plasma cell
abundant RER and well developed golgi apparatus |
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Term
|
Definition
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Term
|
Definition
Th - CD3, CD4
Tc - CD3, CD8 |
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Term
|
Definition
originates from bone marrow stem cells
matures in thymus
majority of circulating lymphocytes = T cells (80%) |
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Term
| Effect of increased HCT on aPTT? |
|
Definition
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|
Term
|
Definition
mutant factor V (R506Q)
cannot be degraded by protein C
prolongs PT/aPTT/TT |
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Term
|
Definition
Mutation in 3' untranslated region
associated with venous clots |
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Term
|
Definition
Inherited (AD)
deficiency of AT III
reduced increase in aPTT after administration of heparin |
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|
Term
| Protein C or S deficiency |
|
Definition
decreased ability to inactivate FV and VIII
increased risk of hemorrhagic skin necrosis following administration of warfarin
K dependent, depleted first with warfarin |
|
|
Term
| What is the first step in platelet plug formation? |
|
Definition
| Adhesion - mediated by vWF, linking of platelet Gp1B receptor to subendothelial collagen |
|
|
Term
| How do platelets aggregate? |
|
Definition
follows adhesion (via vWF-Gp1b interaction)
balance between proaggregation: TxA2 released by platelets
and anti-aggregation: PGI2 and NO released by endothelial cells
Aspirin inhibits COX (and TxA2 synthesis)
Followed by binding of ADP on platelet receptors (insertion of GpIIb/IIIa on platelet membrane allowing for platelet cohesion, Ca strengthens platelet plug) |
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|
Term
| How does vit K participate in the coagulation cascade? |
|
Definition
Activated by epoxide reductase (enzyme inhibited by warfarin)
serves as cofactor to activate precursors of 2,7,9,10,C,S by adding y-carboxyl groups
neonates lack enteric bacteria with produce vit K |
|
|
Term
| What does Warfarin inhibit? |
|
Definition
|
|
Term
| Why are neonates vit K deficient? |
|
Definition
| lack enteric bacteria which produce vit K. |
|
|
Term
| What factors does Antithrombin work on? |
|
Definition
II, VII, IX, X, XI
activated by heparin, anticoagulates |
|
|
Term
|
Definition
Thrombomodulin binds thrombin
activates Protein C
Protein S =cofactor
APC cleaves and inactivates Va, VIIIa
FV leidin mutation = factor V resistant to APC inhibition |
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|
Term
| How does plasminogen work? |
|
Definition
tPA activates is to plasmin
leads to cleavage of fibrin mesh |
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|
Term
|
Definition
Increases vasodilation
increases permeability
increases pain |
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