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Glossary of Block 6 PBL

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B symptoms
Fever, weight loss, night sweats.
Basophilia seen in:
Myeloprolifative disorders (CML, PCV)
Acute leukemias
RBC maturation sequence, time
Proerythroblast
Basophilic normoblast
Polychromatic normoblast
Orthochromatic normoblast
Reticulocyte
Mature erythrocyte

Maturation takes 7 days.

What is it?

Stem cell

What is it?

Proerythroblast

What is it?

Basophilic (early) normoblast

What is it?

Polychromatic (intermediate) normoblast

What is it?

Orthochromatic (late) normoblast

What is it?

Normoblast becoming a reticulocyte by ejecting nucleus. Note reticular network of polyribosomes still there, hence reticulocyte.
Sequence of events in myelopoiesis, maturation length, cell TTL?
Myeloblast
Promyelocyte
Myelocyte
Metamyelocyte
Band
Mature neutrophil, eosinophil, basophil.

Maturation takes 14 days. TTL 1-2 days.

What is it?

Stem cell

What is it?

Myeloblast

What is it?

Promyelocyte - large numbers of peroxidase positive granules.

What is it?

Myelocyte - Losing peroxidase positive granules, getting peroxidase negative granules.

What is it?

Metamyelocyte

What is it?

Band cell

What is it?

Mature Neutrophil
Monocyte maturation sequence
Monoblast
Promonocyte
Monocyte

What is it?

Monoblast

What is it?

Promonocyte

What is it?

Monocyte
Sequence of lymphocyte maturation
Lymphoblast
Prolymphocyte
Lymphocyte (small or large)

What is it?

Lymphoblast

What is it?

Prolymphocyte

What is it?

Large and small lymphocytes
Polycythemia vera: random facts
Effects all 3 lines, reds most
Primary vs secondary
Jak2 gene most common mutation
Longer lifespan, normal replication
Pruritis after hot shower. Gout. Risk of embolic event. Low EPO.
Erythromelalgia
pain in feet or hands due to microvascular thrombi in PV.
4 chronic myeloproliferative disorders
CML
PV
Essential thrombocythemia
Myelofibrosis with myeloid metaplasia
Chemical factors causing myelofibrosis:
Release of PDGF, TGF-beta.
Chronic Idiopathic Myelofibrosis: random facts
Teardrop red cells.
Median survival 3-5yrs.
Extramedullary hematopoesis.
Abnormally large platelets.
No cure, hydroxyurea for palliation.
CML: random facts
BCR from 22 to 9.
Chronic phase to accelerated to blast.
Basophilia, low leukocyte alkphos, high neuts.
Bone marrow transplant or Imatinib
CLL: random facts
Hepatosplenomegaly, hx of infections (hypogammaglobulinemia)
>5000 lymph/mm3 = CLL, else SLL.
Deletions and trisomy, not trans (del-13, tri-12)
CD5+, CD23+, pan Bs (19,20)
No treatment in absence of symptoms.
Multiple Myeloma: random facts
Plasma cell proliferation in marrow
Produce IgG or IgA most often
Renal failure (BJ protein), bone pain, anemia, hypercalcemia, rouleaux
1% nonsecretory, 20% no blood Igs (only BJ proteinuria)
Treat with alkylating agent (cyclophosphamide) + prednisone + bisphosphonates
Hydroxyurea
CML, AML, PV, psoriasis, sickle cell.
Inhibits ribonucleotide reductase -> less purines -> less DNA synthesis. Does not effect protein/RNa synthesis.
Preg class D, causes pancytopenia.
Tamsulosin
Alpha 1 antagonist. Mediate prostate smooth muscle contraction, relaxing prostate and improving urine flow rate in BPH.
MM: staging and treatment
B2 microglobulin, 3.5, 5.5 cutoffs
Also Hg, Calcium, lesions and protein levels.
Treat with conventional chemo, melphalan with stem cell support, or allogenic stem cell transplant.
Plasmaphersis and bisphosphonates too.
Thalidomide
Multiple myeloma
Pro-apoptotic, inhibits growth factors IL6, VEGF. Upregulates NK cells to fight.
Synergistic with dexamethasone (TD therapy)
Risk of DVT. Anticoagulate.
Bortezomib
Inhibitor of 26S proteosome.
Inhibits NF-KB which MM cells use for transcription of growth factors (IL-6, VEGF)
4 types of globulin proteins
Alpha-1: A1AT etc.
Alpha-2: haptoglobin, ceruloplasmin
Beta: transferrin, C3
Gamma: Igs
Polyclonal vs monoclonal
Broad peak in gamma region (infection etc) vs sharp peak in gamma region.
Sodium polystyrene
Cation exchange resin, binds K and releases Na in gut.
Calcium Gluconate
Protects myocardium from hyperkalemia by antagonizing the K+ induced depolarization of membrane potential. No effect on actual hyperkalemia.
Insulin, Albuterol, Furosemide in hyperkalemia
K+ into cells
K+ into cells
K+ out into urine

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