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27 Cards in this Set

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leukocyte/ white blood cells


5 types

1) basophils


2) eosinophils


3) neutrophils


4) monocytes


5) lymphocytes


WBC- total white blood cell count


how many is normal


why is it useful?



differential white cell count (aka "diff")

4,500-10,00


bands 3-5%


useful as both a diagnostic tool and in following the course of disease


if needed, order a differential separately



diff: proportions of different types of leukocytes in the smear of the patient's venous blood

two basic groups of leukocytes

- grandulocytes


neutrophils (or segs) 50-70% relative value (2500-7000 absolute value)


eosinophils 1-3%


basophils: 0.4- 1%



- agranulocytes


lymphocytes 25-35% relative value (1700-3500)


monocytes 4-6% relative value (200-600)

granulocytes/PMNs


neutrophils

the body's primary defense against bacterial infection and physiologic stress


- comprised majority of WBC (60%)


- bands (immature neutrophils) 3-5% of WBC



increased with infections, granuocytic leukemia, burns



decreased with drugs, viral infections, bone marrow invasion or aplasia

granulocytes/PMNs


eosinophils

attack parasites and act as phagocytes


antigen- antibody complexes



increased during an allergic reactions- hay fever, asthma, drug hypersensitivity, parasitic infection, pernicious anemia



decreased with infections, exogenous cortisone

granulocytes/PMNs


basophil

not well understood but similar to mast cells


- secrete anti- coagulant and vasodilatory substances as histamines, heparin, and serotonin


- main function is secreting substance which mediate the hypersensitivity reaction


- possible phagocytory capability



increased with CML, polycythemia, myeloid metaplasia

non-granulocytes

the main constituents of the immune system


- defense against pathogenic micro organisms such as viruses,bacteria, fungi


- T cell matures in thymus gland


- B cell matures in bone marrow


- second most common WBC in adults (neutrophil #1)


- increased during infections, TB, lympocytic leukemia

monocytes

phagocytes and precursors of macrophages


- largest blood cells


- in presence of an inflammation site, monocytes quickly migrate from the blood vessel and start an intense phagocytory activity


increased during monocytic leukemia, TB, collagen disease,chronic infection or inflammation

bands/immature neutrophils

in general, a high number of immature cells in the peripheral blood reflects


- "push on the marrow" to produce cells in large quantities (ie common manifestation of bacterial infection is a shift to the left with high # of immature granulocytes or bands seen on differential)


- developmental arrest suggestign primary hematologic disease

neutropenia


definition


formula

absolute decrease in the number of circulating neutrophils in the blood



absolute neutrophil count (ANC)



ANC = (WBC x (% neutrophils + % bands))/ 100

neutropenia - values for:


mild


moderate


severe

1000-1500


500-1000


<500


<200

diagnostic studies of the hematologic system

- radiologic studies: CT/MRI of lymph tissues


- biopsies


bone marrow exam


lymph node biopsies

leukemia

group of malignant disorders of the hematopoietic tissues characteristically associated with increased numbers of white cells in the bone marrow and/or peripheral blood

classification of leukemias


two origins, acute and chronic


list all four

myeloid orgin, acute is acute myeloid leukemia )(AML)



myeloid orgin, chronic chronic myeloid leukemia (CML)



lymphoid, acute: acute lymphoblastic leukemia (ALL)



lymphoid, chronic: chronic lymhpocytic leukemia (CLL)


causes of acute leukemias

- idiopathic (most)


- underlying hematologic disorders


- chemicals, drugs


- ionizing radiation


- viruses (HTLV I)


- herediaary/ genetic conditions

red flag

- elevated or decreased WBC count with any blasts


- refer to oncology

myeloproliferative disorders


- what are the chronic myeloproliferative disorders?


- what are they characterized by?

- polycythemia vera (PCV)


-essential thrombocytopenia (ET)


- chronic myelogenous leukemia (CML)


- myelofibrosis with myeloid metaplasia



characterized by increased red blood cell mass or erythrocytosis

polycythemia vera


1) incidence


2) clinical features

1) median age: 60 but seen bt 20-85, more in men than women, untreated PCV surival 6-18 months but treated >10 years


2) non specific complaints: HA, weakness, dizziness, excessive sweating, pruritus (after shower, ASA helps), t


- erytromelagia/acra lparesthesias (burning pain/ parathesias in hands/feet with redness, pallor or cyanosis, responds to ASA or reduction in plt count to normal)


- thrombosis (verous or arterial) transient visual disturbances


- splenomegaly +/- hepatomegaly

polycythemia vera


thrombosis

- 2ndary to increased blood viscosity and platelet number, 15% of major thrombotic complication ie CVA, MI, thrombophlebitis, DVT, PE


-denovopresenttion in pts with Budd-Chiari syndromeand portal, splenic, or mesenteric vein thrombosis

polycythemia vera


GI symptoms

- high incidence of epigastric distress, history of PUD, and gastroduodenal erosision on upper endoscopy

polycythemia vera


what do you find on physical exam?

- splenomegaly


- facial plethora (ruddy cyanosis)


- hepatomegaly


- injection of conjuctival small vessels


- excoriation of skin suggesting severe pruritus


- stigmata of prior arterial or venous thrombotic event


- gouty arthritis


- erythromelalgia

erythromelalgia

burning in feet or hands accompanied by erythema, pallor, or cyanosis in presence of palpable pulses


- microvascular thrombic complication in PCV and ET

polycythemia vera


diagnostic criteria

- first r/o secondary causes of erythrocytosis


major


- increased red cell mass: males>= 36, females >= 32


-arterial oxygen saturation = > 92


- splenomegaly



minor criteria:


- platelet count > 400,00/ micro L


- WBC > 12,000 microL


- leukocyte alkaline phosphatase score >100


- vit B12 > 900


requires all 3 major and 2 minor

polycythemia vera


course and prognosis



what's the mean survival?


how long can chronic last?


what can it progress to?


what is the major source of morbidity and mortality

- mean survival is 14 years


- chronic phase may last for years


- progress is myelofibrosis and AML


- thrombosis major source of morbidity and morality

revised WHO criteria for PCV

major:


- Hbg > 18.5 in men, 16.5 g/dL in women


- presence of JAK2 V617F or functionally similar mutation



minor:


- bone marrow biopsy shows hyper cellularity for age with trilineage growth with prominent erythroid, granulocytic, and megakaryocytic proliferation


- serum erythropoietin level below normal reference range


- endogenous erythroid colon formation in vitro

polycythemia vera


treatment

- phlebotomy: reduce viscosity HCT < 45 but increased risk of thrombosis within 3 years leading to + low dose aspirin


- hydroxyurea: non alkalating inhibits enzyme ribonuclotide diphosphate reductase involved in DNA synthesis, reduced incidence of thrombosis compared to phelbotomy


- interferon alpha: anti proliferative and celluar differentiating effects, relieves intractable pruritus and reduces spleen size

polycythemia vera


causes of death

- thrombosis


- hematologic malignancies (AML or MDS)


- non hematologic malignancies


- hemorrhage


- myeloid metaplasia with myelofibrosis