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182 Cards in this Set
- Front
- Back
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When cells are released from the bone marrow, are they usually nucleated or not?
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they are non nucleated (RBC's have no nucleus)
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Where does extramedullay hematopoiesis occur?
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the spleen (1st), liver (2nd), and then other tissues outside the bone marrow
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Whats the life span in days for RBCs?
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120 days
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What type of cell is a myelocyte?
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this is a granulocyte
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What is the normal range for neutrophils?
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1500-6500/ mm3
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What is neutropenia? what level?
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this is a reduction in ALL granulocytes**
below 1500/mm3 |
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What is agraunlocytosis? what level?
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agranulocytosis, granulocytes below 1500/mm3
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What are the causes of neutropenia?
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reduced myeloid stem cell production
drugs causes arrest of granulocyte precursors ineffective |
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What is myelophthisic anemia?
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this is inflitration of something into the bone marrow, causing pancytopenia
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What is aplastic anemia?
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this is a bone marrow precursor failure
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What is the root defect in ineffective graunlopoiesis?
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defective DNA (the messed up chevy plant idea)
can also be B12 or folate |
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What does the blood smear of ineffective granulopoiesis look like?
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these are badly manufactured cells. you see BIG cells, RBCs big
BIG/Hypersegmented neutophils |
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What is the root defect in ineffective graunlopoiesis?
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defective DNA (the messed up chevy plant idea)
can also be B12 or folate |
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What does the blood smear of ineffective granulopoiesis look like?
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these are badly manufactured cells. you see BIG cells, RBCs big
BIG/Hypersegmented neutophils |
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What immune mediated things can increase neutropenia/ accelerated destruction/increased use
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SLE or idiopathic antibodies
drug reactions Increased use due to Overwhleming infections** |
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What drugs cause neutropenia? (below 1500)
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alcohol**
sulfonamides, thiouracil chemotheraphy |
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What infections are common in neutropenia?
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Aspergillus
Candida |
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what is agrunlocytic angina?
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ulceration/necrotizing lesions of the gingiva, mouth, pharynx
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What the best test to differentiate the different pancytopenias?
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bone marrow biopsy
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What are the defining levels for lymphocytopenia in adults and children?
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adults less than 1200
kids less than 3000 |
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What are the main causes of Neutrophilia?
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bacterial infections
inflammatory rheumatic fever neoplasias |
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What pool do neutrophils come from? under what conditions?
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the margination pool- releasing mature segmented neutrophils
Exercise and catecholamines |
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What things cause an increase in release of neutrophils from bone marrow storage pools?
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acute infection
hypoxia endotoxin cytokines |
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What happens to the # of band cells in neutrophilia?
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they will increase greatly
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What morphologic changes occur in neutrophils with severe inflammation? (3)
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Toxic granulation
Dohle bodies cytoplasmic vaculoes |
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What do Dohle bodies look like?
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this is blue staining dilated ER
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What does toxic granulation look like?
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coarse dark cytoplasmic granules, primary granules
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What do neutrophils due to severe inflammation due to?
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bacterial infection
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What is a leukemoid reaction?
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this is high granuloctyes, usually above 50,000
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What causes a leukemoid shift?
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inflammation, infection, tumors
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What is a "left shift"
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this is increased bands, metamyelocytes
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What is a leukoerythroblastic reaction?
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increase in immature RBC's, metamyelocytes, granuloctyes
due to infections or other causes |
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What is the germ line cells for WBC's?
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granulocytes
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What cytokines (two) stimulate granulocyte proliferation
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IL-1 and TNF-alpha
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What defines a leukoerythroblastic reaction?
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presence of nucleated RBC's and immature neutrophils in peripheral smear
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What causes leukoerytrhoblastic RXN?
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usually a space occupying lesion of the bone marrow
(tumors and such) |
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What does a high neutrophil count during an MI indicate?
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this indicates tissue damage, neutrophils infiltrate the heart muscle after damage
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What is the normal range and percent for eosinophils?
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0-400, or 0-1% normally
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What do eosinophils respond to?
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allergies and parasite infections
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What stimulates eosinophilia?
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IL-5 stimulated differentiation of precursor cells ***
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What causes eosinophilia to occur?
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allergies, parasites, allergic drug reactions
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What defines eosinophila?
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absolute eosinophils greater than 400 (or about 1%)
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How do you rapidly decrease eosinophil counts?
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corticosteroids
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What do charcot leyden crystals indicate?
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these indicate eosinophila
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What causes monocytosis?
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chronic infections**
like TB, malaria, subacute bacterial endocarditis, IBS, lymphomas, SLE, RA |
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Case 7#
high WBC Low platelets, low Hb/Hct, +3 macrocytes with polychromasia icterus, brusing + DAT leads you to what diagnosis? |
this shows blood loss anemia, probably due to destruction of cells by antibodies
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What are the chief signs of Mononucleosis
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Enlarged cervical lymphnodes
maculopapular rash high WBC high lymphocytes and Reactive/atypical lymphocytes*** |
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What is the significance of atypical lymphocytes?
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VIRAL infection***
these usually occur in mononucleosis- due to CD8+ |
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What is a heterophile antibody used to diagnose?
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mononucleosis aka mono spot test
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What two other diseases can cause mono like lymphocytosis?
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CMV
toxoplasmosis |
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What are the causes of nucleated RBC's in the peripheral blood
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rapidly evolving anemias
post splenectomy bone marrow replacement diseases |
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What cells are implicated in lymphoid neoplasms?
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B cell, T cell, NK cells
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What are the common signs of acute leukemias?
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fatigue
fever-infection petechiae/bleeding bones painful to pressure |
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What does the bone marrow look like for acute leukemias?
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Hypercellular, with greater than 20% Blasts (primitive cells with large nuclei)
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What is the peak age for ALL?
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4 years old, most under 15
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What is the peak age for AML?
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15-39 years old
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What are the specific tests for ALL?
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TdT positive, PAS positive
80% pre B: with CD10 and CD19 can be Pre T with CD7, CD2, CD3 |
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What are the specific tests for AML
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Myeloperoxidase positive
Auer Rods |
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how do glucocorticoids affect the CBC?
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drop eosinophils/lymphoctyes
increase neutrophils (glucocorticoids dont like neutrophils leave the blood stream) |
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what kinds of hypersensitivity reaction leads to eosinophilia
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type 1 hypersensitivity
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what kind of tosis do graunlomatous diseases cause?
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monocytosis
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What do ballerina skirt lymphoctyes indicate?
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these are for atypical lymphs that are CD8+ and fighting viral infections
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in ALL, what are the markers for it being a Pre-B type?
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TdT, CD10, CD19
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In ALL, what are the markers for it being a Pre-T type?
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TdT, CD1, CD2, CD3, CD7
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What does TdT show?
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this is the ABSOLUTE marker for a lymphoBLASTIC leukemia
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what is the most common type of ALL?
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Pre-B (has TdT and CD10)
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What is the presentation of T lymphoblastic lymphoma?
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mediastinal mass involving thymus
respiratory symptoms |
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Who is T lymphoblastic lymphoma most common in?
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adolescent males
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What is the prognosis for T lymphoblastic lymphoma?
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this is a bad prognosis
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What are the lab findings in ALL in regards to the bone?
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bone marrow is greater than 20% BLASTS
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What are the buzz words to look for in the bone marrow that lead you to ALL?
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bone marrow replacement with Primitive Cells
Delicate Chromatin Nucleoli CD10 (CALLA) TdT |
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What genotype of ALL has a good outcome?
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hyperploidy is good
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What genotype of ALL has a POOR prognosis?
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t(9:22) philadelphia chromosome
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What is the prognosis of ALL without therapy?
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they will die in less than 3 months
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How good are treatment outcomes of ALL?
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95% remission in kids
and 75% cured in kids |
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What are the best prognostic factors for ALL?
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young with pre-B type
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What are found in blasts of AML?
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auer rods , these are spikey rods found inside myeloblasts
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what is the age of AML?
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15-39
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What AML abberations are associated with a poor prognosis?
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myelodysplasia like abberations of 5q, 7q, 20q
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What AML cause has a VERY poor prognosis?
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post prior chemotherapy AML is very bad.
typically after toposiomerase II |
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What are the features of M2 AML?
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BLASTS with AUER rods
Stains with myeloperoxidase |
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What is the most common type of AML?
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M2 acute myeloBLASTIC leukemia
Blasts with auer rods |
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What are the features of M3 AML?
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Auer rods
Alot of GRAULES*** Promyeloctes |
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What trans-location causes M3 AML?
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t(15:17) PML- next to the retinoic acid receptor.
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What are the treatments for M3 AML (acute promyelocytic leukemia)
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treat with retinoic acid to prevent DIC
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What is the diagnostic test for AML?
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>20% BLASTS in bone marrow
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What disease does Chloroma suggest?
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AML- this predicts that AML will develop soon if not currently present
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What is the prognosis of AML?
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60% remission
15-30% disease free at 5 years |
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What is the standard age and gender for CLL/SLL?
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median age of 60
males most common |
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What are the CD makers for CLL/SLL?
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CD19,CD20, CD23 (B cell markers) PLUS CD5***
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What CLL subtype is a good prognosis?
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Zap-70 NEGATIVE, somatically hypermutated cells
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What CLL subtype is a POOR prognosis?
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Zap-70 POSITIVE is poor
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What cells on peripheral smear suggest CLL?
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Smudge cells , with essentially normally size lymphoctyes
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What do spherocytes indicate?
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hemolysis
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how long do you live with CLL?
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4-6 median, can live more than 10+
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How do CLL pts appear?
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they appear healthy overall
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What are the general signs of CML?
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Left shift
Palpable spleen No anemia increased platelets |
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What is the main problem in CML?
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these are too many myelocytes of various kinds
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When do people usually get CML?
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25-60 years old
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What is the molecular basis of CML?
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BCR-ABL translocation (see with fish)
t(9,22) philadelphia chromosome |
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What does ABL normally do?
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this causes cell division via a Jak/Stat pathway
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What Lab findings with CML?
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Neutrophilia is ALWAYS present
may also have eosinophila/basophila but BLASTS are NOT super high like acutes |
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What is the clinical presentation of CML?
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slow onset
splenomegaly- heavy abdomen, early satiety, LUQ pain night sweats |
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How does CML progress?
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this turns into an accelerated phase, which turns into a blast crisis (with AML or ALL)
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What is the Tx of CML?
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long term control with Imatinib (Gleevic)
a BCL-ABL antagonist |
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What type of cells cause Hairy cell leukemia?
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B cell neoplasms
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Who usually gets HCL?
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middle aged men
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What are the markers for HCL?
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B cell makers like CD19, CD20
and a monocyte associated antigen like CD11c, CD22 |
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What are the clinical signs of HCL?
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splenomegally, typically massive
pancytopenia from marrow replacement high rate of mycobacterial infections |
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How well do people recover from HCL with Tx?
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95% survive 5 years or more
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are lymph nodes enlarged in HCL?
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no they are not** kind of fun fact
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What does myelodysplasia mean?
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this is PRE cancer of myeloid stem cell line
these show as cytoPENIA** cells are DYSFUNCTIONAL** |
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What is the defect that leads to Myelodysplasia syndromes?
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this is caused by stem cell genetic damage
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What does therapy related MDS often turn into?
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early transformation in AML is likely
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What does the morphology of MDS look like?
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Pancytopenia
MCV is large Giant Platelets Hypercellular marrow*** Ringed Sideroblasts*** |
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What is the usual clinical features of MDS?
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anemia or pancytopenia
weakness infections and bleeding |
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How long is the survival of MDS?
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only about 5 years tops
median is 9-29 months usually dies due to acute leukemia |
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What is the clinical presentation of PCV (poly cythemia vera)
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Purpura, headaches, abdominal fullness
Splenomegaly elevated hemoglobin, elevated platelets elevated WBC |
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is PVC independent of EPO?
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yes it is, it does not need growth factors
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What mutation causes PVC?
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this is a point mutation in JAK2 hematopoietic growth pathway
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What does the clinical course of polycythemia vera look like?
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Plethoric- with excess of body fluids
has a cyanotic complexion splenomegaly HTN headache peptic ulcer and intense pruritis |
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What are the lab findings in polycythemia vera?
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Increased hemoglobin
high Hct very LOW EPO levels High WBC's High platelets |
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what is essential thrombocytosis?
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this is platelets above 600,000 in the absence of any other myeloproliferative disorders
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what are the clinical signs of essential thrombocytosis?
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thrombosis and hemorrhage
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What are the labs for essential thrombocytosis?
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Large platelets in smear- and too many of them***
increased megakaryoctes platelet functional deficits (they make too many too large and they dont work) |
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What are some causes for reactive thrombocytosis?
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acute infection/inflammation
chronic inflammation asplenism blood loss anemia |
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What is primary myelofibrosis?
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this is a clonal neoplastic transformation of myeloid stem cells.
fibrosis of bone marrow, followed by hematopoiesis in other organs*** |
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what mutation causes myelofibrosis?
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JAK2 mutation,
megakaryoctyes release fibrogenic factors PDGF and TGF-B |
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What is the clinical presentation of Myelofibrosis?
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fatigue, weight loss
MASSIVE spleen** Hyperuricemia extramedullary hematopoesis |
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What are the lab findings in myelofibrosis?
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Leukoerythroblastosis
Tear drop erythrocytes** NRBC (nucleated red blood cells) anemia Giant platelets** |
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How do we usually get lead in out bodies?
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inhalation (occupational)
or ingestion in food and liquids |
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How is lead toxic (biochemically)
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this inhibits the enzymes involved in incorporating iron into heme.
delta aminolevulinic acid dehydratase is inhibited. Ferrochetalase |
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What reaction does ferrochelatase do?
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this turns protoproporhyrin into heme by adding Fe2+
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Other than messing with heme synthesis, lead is toxic in other ways, what are they?
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competes for calcium ions (gets stored in bones)
inhibits membrane associated enzymes= Hemolysis*** impairs vitamin D synthesis |
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What are the MAIN blood signs of lead toxicity ***?
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Hypochromic microcytic anemia with Basophilic stippling
increased serum iron |
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what re the kidney signs of lead exposure?
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gout (renal injury)
HTN chronic tubulointerstitial disease |
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What are they key morphological features of Langerhans Cell Histiocytosis?
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Birbeck Granules
high CD1a expression vesicular nuclei with linear grooves |
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What age is Letterer siwe disease common?
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most under age 2
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What are the signs of Letterer siwe disease?
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skin rash
hepatosplenomegaly, lymphadenopahy anemia,thrombocytopenia |
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What is the root cause of Letterer siwe disease?
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acute disseminated langerhans cell histocytosis
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What is an Eosinophilic granuloma
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this is langerhans cells mixed with eosinophils, lymphs, plasma cells, and PMNs
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Where are Eosinophilic granulomas usually located?
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bone, medullary cavity, calvarium, ribs, femur
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What is the prognosis with eosinophilic graunlomas?
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these may regress spontaneously, or may need local excision
the prognosis is good |
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What is Hand-Schuller- Christian triad?
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eosinophilic granulomas of calvarial bone (the skull)
Exopthalmos Diabetes insipidus |
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What are plasma cell neoplasms?
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monoclonal gammapathy, with M protein or component being very high
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What is the most common type of plasma cell neoplasm?
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monoclonal gammopathy of undetermined significance (MGUS)
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What is multiple myeloma?
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this is where plasma cells form tumors at multiple sites within bone marrow
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What are the main causes of multiple myeloma?
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strong genetic component- blacks
ionizing radiation chronic inflammation |
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What interleuken is elevated in multiple myeloma?
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IL-6
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What is the pathology of multiple myeloma? what does it do to the body?
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this causes lytic bone destruction
excess immunogloblin production causes- increased serum viscosity, RENAL damage**, suppresses normal immune system |
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what are the Xray findings of multiple myeloma?
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punch out osteolytic defects of the bone, vertebral column, ribs and skull
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What happens to the kidney in multiple myeloma?
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Bence jones light chain protein precipitates in the tubules forming eosinophilic casts**
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What are the blood findings in multiple myeloma?
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Bone marrow plasmacytosis, too many plasma cells and plasmablasts (and abnormal ones at that)
Russell bodies** (looks like bubbly pancake batter) |
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What are the clinical signs of multiple myeloma?
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bone pain
hypercalcemia reccurent infections renal insufficiency amyloidosis hyperviscosity syndrome |
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What are the lab findings for multiple myeloma?
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M protein
bence jones protein in urine (kappa/lambda light chains) Rouleaux (the stacked RBC's) |
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What is the diagnostic criteria for Multiple myeloma?
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Plasmacytosis in +10% of bone marrow
Monoclonal protein in serum/urine (m protein in serum, bence-jones protein in urine) End organ damage with! ( CRAB- hypercalcemia, renal insufficiency, anemia, bone lesions) |
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What is the treatment for Multiple myeloma?
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palliative care only- can extend life a couple years with transplants and alkylating agents
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What is a solitary plasmacytoma?
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this is a localized tumor of plasma cells
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Can solitary plasmacytoma lead to multiple myleoma?
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yes it can, but many years down the road
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Other than bones, where can you find solitary plasmacytomas?
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URT
sinuses lungs oropharynx |
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What is MGUS (monoclonal gammapthy of uncertain significance)
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this is M protein found in an ASYMPTOMATIC** person.
NO other signs of myeloma |
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What is the most common monoclonal gammapthy?
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MGUS
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what is the significance of MGUS?
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this is precancerous
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What age group usually gets lymphoplasmacytic lymphoma?
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this is b cell neoplasm of people over age 50
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What is the morphology of lymphoplasmacytic lymphoma?
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this involved lymph nodes, liver and spleen
B cell markers CD19/CD20 are elevated High monoclonal immunoglobulins |
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What are the clinical features of lymphoplasmacytic lymphoma?
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lymphadenopathy
hepatosplenomegaly anemia from bone marrow replacement*** autoimmune hemolytic anemia from COLD agglutinins*** |
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What disease causes Waldenstroms macroglobulinemia?
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lymphoplasmacytic lymphoma
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What are the signs of Waldenstroms macroglobulinemia
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bleeding
cryoglobulinemia raynauds neuro symptoms sluggish blood flow |
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What kinds of things does the spleen filter out of the blood?
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Unwanted elements from RBCs like:
old RBCs howell jolly, heinz bodies encapsulated bacteria macromolecules cell debris |
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What type of immunity does the spleen help?
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the humoral immunity
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what types of cells does the spleen store, and how much of them?
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30-40ml of RBCs
30-40% of platelets |
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What are the causes of splenomegaly?
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infections
congestion blood disorders immune-inflammation GSD's |
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What are the symptoms of splenomgealy?
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dragging/heavy LUQ
discomfort with eating early satiety |
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what two infections commonly involve the spleen?
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mono
CMV |
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how do myeloproliferative disorders affect the spleen?
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the spleen is ALWAYS massive in these diseases
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What is the hypersplenism triad?
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splenomegaly
reduction in one or more blood elements compensatory bone marrow hyperplasia |
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What are the causes of splenic infarcts?
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emoboli from the heart
sickle cell anemia DIC splenomegaly |
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What are the symptoms of splenic infarcts
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sharp LUQ pain
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What two cancers metatases to the spleen?
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malignant melanoma
lung cancer |
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What is the only primary cancer of the spleen?
|
lymphohematopoietic malginancy
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What is the post splenectomy CBC like?
|
NRBC's
Howell-Jolly bodies target cells (plus infection with encapsulated organisms) |
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what are the signs of a ruptured spleen?
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massive intraabdominal hemorrhage
hypovolemic shock tx- surgery! |
