6. White Blood Cell disorders Pathoma Flashcards
What are some causes of Neutropenia
Sepsis/severe infection
Drugs (chemotherapy)
What are causes of Lymphopenia
Immunodeficiency: HIV, SCID, SLE, sepsis
High levels of cortisol (or corticosteroids)-these cause apoptosis
Radiation (lmyphocytes die quickest)
Whats the anatomy of lymph node
What areas are the B and T cells located
B cells are in the cortex (follicles)
T cells are the in the Paracortex (swells with viral infection)
What are the markers for Lymphoblasts in the blood?
Myeloblasts?
TdT+ (marker of pre T and pre B)
CD10,19, 20 (marker of pre B)
_______________________
myeloperoxidase(+)
Auer Rod
Tell me about ALL (acute lymphoblastic Leukemia)
whats the good translocation
Most frequently in childeren
Highly associated with Down syndrome (after age of 5)
T(12;21) has good prognosis
Subdivided into T-ALL and B-ALL
**acute is always immature
T-ALL what physical finding should give it away
T should make you think of Thymic (Mediastinal mass)
AML (Acute Myeloblastic leukemia)
whats the classic translocation (also known as APL-acute promyelocytic leukemia). and what does this trans do?
what systemic problem can AML lead to? and how do you treat it?
Myeloperoxidase (+) ->these aggregate to form Auer Rods
APL- T(15;17) fucks up the retinoic acid receptor ->the cells cant mature
can lead to DIC (treat with ATRA-causes blasts to mature)
under AML, if the leukemia is specifically monocytes, what should you think of
speciffically megakaryocytes
lack Myeloperoxidase//heavily infiltrates the Gums
Associated with Downs syndrome before 5
whats the deal with CLL
who does it affect, what are the cell markers
what type of cells will histo show
what can CLL transform into?
Usually in older adults
proliferation of naive B cells (CD5,20)
smudge cell
Diffuse Large B-cell Lymphoma (DLBCL)
Hairy Cell Leukemia
what does it stain positive for?
clinical features
proliferation of Mature B-cells
hairy/fuzzy cells
stains positive for TRAp
massive Splenomegaly (red pulp) / dry tap on bone marrow aspiration
CML (chronic myelogenous leukemia)
whats the translocation
how do you treat it
what can it transform to?
increase of mature granulocyte (mostly basophils) LAP (-)
Philadelphia chromosome t(9;22)
BCR-ABL (increases tyrosine kinase activity)
To treat, you gotta inhibit tyrosine kinase - Imatinib
can transform into Acute Leukemia
Polycythemia Vera
what is messed up(mutation) that causes it
clinical symptoms
Treatment?
proliferation of mature myeloid cells (especially RBCs) EPO is decreased
JAK2 kinase mutaiton
-blurry vision, Increased risk of thrombosis (Budd chiari), flushed face, itching after bathing (mast cells release histamine)
tx-phlebotomy (get rid of some of that blood)
2nd tx is hydroxyurea
2° Polycythemia (reactive)
Increased EPO from either lung dz (low O2)
or Tumor that produces EPO (renal cell carcinoma)
Essential Thrombocythemia
proliferative disorder of myeloids (specifically Platelets)
shit ton of platelets - either bleeding or thrombosis from lack of function or overfunctioning platelets
Myelofibrosis
what does it lead to
what cells does it make
myeloproliferative disorder (specifically Megakaryocytes)
JAK2 kinase mutation
marrow fibrosis - so the marrow cant make new RBCs which means you get extramedullat hematopoeisis in the spleen (splenomegaly)
Teardrop cell
Lymphoma
What are the subcategories
Tumor that arises from lymphocyte
Hodgkin lymphoma &
Non-Hodgkin Lym : Burkitt, DLBCL, Mantle,
Follicular Lymphoma
what is it/clinical signs
translocation
NHL
neoplastic Bcells (CD20) that make small follicle like nodules
Painless LAD (waxing and waning)
T(14;18) - switches heavy chain IG with BCL-2 (inhibits apoptosis) so you get over exp resion of BCL-2
What does BCL-2 do
cell marker that inhibits apoptosis
Mantle Cell Lymphoma
whats the translocation
neoplastic B cells that expand the mantle zone
t(11;14) cyclin D and IG Heavy chain
overexpression of cyclin D causes cell to go from G1 to S phase
What does Cyclin D do?
causes cell to go from G1 to S phase in cell cycle
Burkitts Lymphoma
What is it associated with
what are the 2 different forms
Translocation/ what does histo look like
neoplastic B cell prolif (CD20)
associated with EBV
-Endemic African: Jaw lesion // Sporadic: abdomen
t(8;14) : C-myc (promotes cell growth)
Starry Sky Appearance
Diffuse Large B-cell Lymphoma (DLBCL)
most common form of NHL
large nodes or extranodal mass
Hodgkin Lymphoma
what cell do you see on histo
Reed-sternberg cells (owl eyes) CD15/30
you get B-symptoms: fever, night sweats, weight loss
Multiple Myeloma
what are some sypmtoms (CRAB)
which IG’s are most spiked
associated with
Malignant proliferation of plasma cells in marrow
Fried egg appearance
HyperCalcemia, Renal involvment, Anemia, Bone lesion (lytic- punched out)
M Protein spike (increase in Monoclonal immunoglobin (IG G/A)
Increased risk of infection, Rouleaux formation (stacked RBCs), Ig light chains in urine
