Term
anemia Hb level?
Sxs of anemia (7)?
First test you order if you see an anemia?
Subdivisions of anemia? |
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Definition
anemia w/ hb <12. aging does not cause anemia and is not normal
Sxs - palor, pica, orthostatics, palpitations, chest pain, dyspnea on exertion, and edema
Sub divisions - hyperproliferative and hypoproliferative based on retic count |
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Term
Hyperproliferative anemia causes?
Presentation according to cause? |
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Definition
Bleeding -> either from obvious source or internal source. A/w orthostatics and hypotension
Hemolyzing -> G6PD and PK deficiency, HbS, and Hereditary spherocytosis. Need to order combs test to guide diagnosis!!! |
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Term
| Causes of coumbs positive Hemolytic anemia? Sxs and Tx of each? |
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Definition
IgG causes
SLE - can make IgM Abs too
IAHA
Drugs - Penicillin
CLL, HIV
IgM
mycoplasma infections - usually ansymptomatic.
Treat Coumbs + HA -> steroids, splenectomy, folic acid, transfusions. |
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Term
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Definition
G6PD and PK deficiency
HbS and other hemoblobinopathies
Hereditary Spherocytosis |
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Term
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Definition
Pt only makes spheres. will see very very little donuts. Rare disease
Tx - folate support and early spleenectomy |
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Term
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Definition
sickle cell disease
Tall and skinny w/ long legs. Almost never see a fat HbS pt
Sxs - sclerodactly, painful crisis, non-healing ulcers, pneumococcal infections, chest wall syndrome, stroke, aplastic crisis, priapism and cholelithiasis
Exacerbated by folate defic, parvo
Tx - hydrate, hydroxyurea, exchange transfusion |
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Term
| causes of hypoproliferative anemia? |
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Definition
Low MCV - iron defic anemia, Thallasemia, Sideroblastic Anemia, Endocrinopathies
Normal MCV - combination disorders, paraproteinemia, ACD
Macrocytic - B12 or folate def, drugs, myelodysplasia |
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Term
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Definition
Fe low, TIBC high, Ferritin low (best test), transferrin saturation low. Must find etiology
Bleeding, inability to absorb iron (celiac, gastric bypass, crohns), diet |
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Term
Anemia of chronic disease
Key differentiator? |
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Definition
Low Fe and Low TIBC!!!
ferritin is normal or high
common causes are rheumatic fever or hypothyroid
No treatment usually needed if you cure the underlying disorder. Exogenous erythro may halep |
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Term
Indications for epoitin alfa use? 5
Risks of use? |
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Definition
chronic renal failure anemia
HIV treatment anemia
Chemotherapy anemia
Reduce transfusions
anemia of chronic diseases
myelodysplastic anemias
Risks -> thrombosis or worsen renal disease if Hct rises too high |
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Term
| B12 deficiency 8 causes? Tx? |
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Definition
| chronic pancreatitis, overgrowth of bacteria, IBD, gastric bypass, pernicous anemia (MC), atrophic gastritis, vegans, Drugs )cholestyramine, metformin, colchicine) Supplement - 1000 mcg qd x 7 --> 1000mcg weekly x 4 weeks -> 1000 mcg monthly |
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Term
| Define a myeloproliferative disorder |
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Definition
| a disorder of overproduction of contents of the bone marrow. Components are red blood cells, platelets, stromal tissue, white blood cells. |
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Term
| what is relative polycythemia? causes? |
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Definition
caused by a contraction of the plasma volume thus falsely elevating the Hct.
Causes: diuretic therapy, heat exposure and dehydration, Gaisbocks Syndrome |
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Term
| What is Gaisbocks syndrome? |
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Definition
| a relative polycythemia that is typically in a young executive that smokes and is stressed. Once again, just dehydration. |
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Term
| What is secondary polycythemia? 5 Causes? Tx? |
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Definition
it is a true polycythemia in which the plasma volume is normal w/ an increased Hct. But it is a reaction to something, not a primary bone marrow abnormality.
Causes - Cardiac abnormalities, Pulmonary abnormalities (COPD, smokers), sleep apnea, Renal disease, Hepatoma
Tx - hydroxyruea and phlebotomy until the pt feels good. Not to a certain level |
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Term
Polycythemia Rubra Vera
Sxs? Diagnosis? Complications? Tx? |
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Definition
Lethargy, fatigue, plethora, stroke (often presenting symptom), erythromelalgia, palmar erythema, HTN, Splenomegaly, epistaxis, GI or mucocutaneous bleeding
Dx - Major -> Hb >18.5 men and 16.5 women, JAK2 Mutation. Minor -> hypercellular bone marrow, low ERP, endogenous RBC colony formation on culture
Complications - spent phase, transform to acute leukemia
Tx - keep the viscosity down. Hydroxurea, phlebotomy. |
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Term
Essential Thrombocytosis
Sxs? Dx? Tx? |
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Definition
A diagnosis of exclusion. Rule out Fe deficit, inflammatory conditions, malignancy and infection first.
Sxs - vasomotor (headaches, visual changes, paresthesias, dizzy), thrombotic (TIA, CVA, Portal clot), Hemorrhagic (GI, mucosal, epistaxis). No age association
Dx - platelets >450, marrow w/ proliferation of megakaryocytes, r/o PRV, AMM, CML, MDS
Tx - goal is to prevent complications and treat according to how they feel. Use hydroxyurea, or interferon in preg pts, aspirin, heparin, and coumadin to prevent thrombus |
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Term
Agnogenic Myeloid Metaplasia (AMM)
aka Primary Myelofibrosis
Sxs? Dx? Tx? |
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Definition
blood tests don't tell you much b/c they can vary
Sxs - Pancytopenia, Massive splenomegaly from extramedullary hematopoiesis may cause early satiety and abd discomfort, fatigue, weight loss, anemia sxs, esophageal varicies/bleeding from splenomagaly and portal HTN, bleeding/bruising from thrombocytopenia, hyperuricemia/Gout from cell turnover
Complications - splenic infarcts and portal HTN
Dx - blood smear shows leukoerythroblastic changes (nucleated RBC, tear drops), and bone marrow is dry tap. Stain BM w/ reticulin to confirm fibrosis.
Tx - poor prognosis. Transfusion support, JAK2 inhibitors |
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Term
CLL
Epidemiology?
sxs?
Complications?
Dx? |
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Definition
higher in whites, and >never <40. abnormal prolif of B Cells w/ T markers. Typical B symptoms w/ abd discomfort, early satiety (splenomegaly), LAD, fatigue
Complication: infection is MCC of death. resultant from hypogammaglobulinemia and complement dysfunction -> pulmonary infxns (including opportunistics once immunosuppressed), and transform->Richters syndrome or prolymphocytic leukemia, AHA, and ITP
Dx: often incidental on CBC count. high absolute lymph count, monoclonal surface Ig and CD5+/CD19 |
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Term
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Definition
CLL transforms into a large cell lymphoma
sxs - increasing LAD, HSmegaly, fever, abd pain, weight loss, anemia, thrombocytopenia, high LDH |
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Term
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Definition
| anemia, thrombocytopenia, high prolymphocytes in peripheral smear, LAD, wasting, |
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Term
| Autoimmune complications of CLL |
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Definition
may have a coumbs positive hemolysis anemia and immune thrombocytopenia.
Both respond to steroids, IV Ig, rituximab or splenectomy |
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Term
CLL prognostic factors
Genetics?
Tx? |
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Definition
Bad prognosis: advanced stage, short doubling time, bone marrow infiltration, older males, high B2-microglobulin, Soluble CD23.
Tx - alkylating chemo, purine analogs (fludrabine). Rituximab, alemtuzumab to treat the immune problems.
However, it is incurable. Treat the symptomatic. |
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Term
Chronic Myelogenous Leukemia
Epidemiology?
Sxs?
Course? |
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Definition
A clonal proliferating mature neutrophil disorder. Occurs in any age group. MC elderly
WBC's are are mature and function thus infections not common. causes anemia -> fatigue. Splenomagaly -> abd discomfort. B symptoms. Many are asymptomatic.
t(9;22) BCR-ABL fusion.
Course: chronic 4-6 years, acclerated, and blast phase (acute leukemia) |
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Term
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Definition
Gleevec aka imatinib
a tyrosine kinase inhibitor that revolutionized treatment
Possible to have mutation causing resistance. esp if inadequate dosing or interrupted therapy
2nd gen - nilotinib, dasatinib, bosutinib
Sooner dx is made and treatment started the better outcomes |
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Term
Multiple Myeloma
Sxs? Dx?
Prognosis?
Epidiemiology? |
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Definition
Sxs - Lytic bone lesions, fatigue weight loss, arthritis, kyphoscoliosis, edema, mild anemia, infections, hypercalcemia, elevated creatinine, elevated total protein
SPEP shows Ig spike (MC IgG), bence jones proteinuria, flow cytometry shows decreased kappa:lambda (increased lambda), x-ray lytic lesions, rouleux RBC formation d/t Abs. Confirm w/ bone marrow biopsy
affects the elderly and AA.
Incurable. High LDH and B2-microglobulin = bad |
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Term
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Definition
an aggregation of multiple myeloma plasma cells that form in an organ somewhere. emergency if neuro involvment
Causes - spinal cord compression, |
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Term
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Definition
incurable. Treatment is aimed at control via plasmacytosis using chemo aklylating agents, thalidomide, revlimid
Can use thalidomide to improve response rates of chemo and steroids.
revlimid |
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Term
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Definition
| derivative of thalidomide, more potent though. No neuropathy or sedation. |
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Term
| Complication of Multiple myeloma |
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Definition
Infections. anemia b/c of the kidney disease or the myeloma.
renal insufficiency due to damage from hypercalcemia. limit contrast dye use
Lytic lesions - treat w/ bisphosphonates
rare may progress to osteonecrosis |
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Term
Hodgkins Lymphoma
Sxs? epidemiology? RFs? Labs? Dx? |
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Definition
Potentially curable. MC lymphoma in young adults. Bimodal age distribution
Present w/ LAD, HSmegaly, A symptoms (aka no sxs) or B sxs
CBC will be normal. Increased LDH
dx - you need a lymph node excision w/ biopsy. Shows reed sternberg cells
RF - EBV, HTLV-1, altered immunity, Wiskott Aldrich, HIV, herbicides, pesticides
4 categories: lymphocyte predominant (best prognosis), lymphocyte depleted, nodular sclerosing, mixed cellularity |
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Term
Non-Hodgkin Lymphoma categories and specific diseases.
Tx? |
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Definition
can be indolent, intermediate, or high grade.
Indolent - follicular lymphoma, Small lymphocytic lymphoma
Intermediate - large B Cell lymphoma
High grade - burkitt lymphoma
Tx - radiation therapy for bulky disease and ABVD chemo. Combine them to limit radiation and chemo affects |
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Term
| Follicular lymphoma and SLL |
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Definition
| slow growing non-hodgkins lymphoma. Since it is slow, chemo is ineffective. Not curable but well controlled |
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Term
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Definition
intermediate non-hodgkin lymphoma
The MC non-hodgkin lymphoma.
Effectively treated and cured w/ CHOP chemo |
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Term
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Definition
high grade lymphoma w/ t(8;14) translocation. c-MYC gene w/ IgH gene.
Results in massive lymph nodes w/ rapid growth.
A/w EBV
Needs aggressive treatment. |
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