Deconstructing the CBC - PDF Free Download

Deconstructing the CBC Dr. Ann M. Wexler Solano Hematology Oncology September 10, 2017

What Are the Major Components of Blood? Red Blood Cells (also called erythrocytes) White Blood Cells (also called leukocytes) Platelets (also called thrombocytes) Plasma 2

Fancy Words for High and Low Cell Type Too High Too Low Red Blood Cells (Erythrocytes) Platelets (Thrombocytes) Erythrocytosis or Polycythemia Thrombocytosis or Thrombocythemia Anemia Thrombocytopenia White Blood Cells (Leukocytes) Leukocytosis Leukopenia Neutrophils Neutrophilia Neutropenia Lymphocytes Lymphocytosis Lymphopenia Eosinophils Eosinophilia --- Monocytes Monocytosis --- Basophils Basophilia --- 3

Red Blood Cells (RBCs) Largest cellular component of blood, about 40-45% of blood volume Comprised mostly of hemoglobin Transport oxygen Red blood cells normally last about 120 days before they are removed by the spleen 4

Hemoglobin Red pigment molecule which gives RBCs (and blood) its color Contains 4 molecules of heme and 4 of globin (2 alpha chains and 2 beta chains) Each molecule of heme contains one iron ion 5

White Blood Cells There are several types of white blood cells They are all involved in immunity but in somewhat different ways Granulocytes (neutrophils, PMNs, polymorphonuclear leukocytes, or polys ) involved in acute infections with bacteria. Immature form is called a band Lymphocytes involved in many types of infection, especially viruses - produce antibodies and memory cells - are further divided into T-cells and B-cells Eosinophils involved in parasitic infections and allergies Basophils involved in parasitic infections and allergies Monocytes involved in bacterial and parasitic infections 6

White Blood Cells (WBCs) Neutrophils also called a variety of other names on CBC reports, including: polys PMNs segs grans Lymphocytes Monocytes Eosinophils Basophils 7

Platelets Really more of a fragment of a cell They are broken off from a very large cell in the bone marrow called a megakaryocyte Primary function is to aid in blood clotting Lifetime in the blood is 7-10 days after which they are destroyed in the spleen 8

Plasma Plasma is the liquid component of blood Comprised mostly of water, but also includes: Protein (albumin, globulin, fibrinogen) Lipids (cholesterol, triglycerides) Dissolved salts and minerals (sodium, calcium, potassium) Glucose 9

Normal Peripheral Smear

Complete Blood Count (CBC) TESTz RESULT UNITS REF RANGE WBC 5.2 x 1000/mm 3 3.9-11.1 RBC 3.81 L x 10 6 /mm 3 4.20-5.70 HGB 14.5 g/dl 13.2-16.9 HCT 41.2 % 38.5-49.0 MCV 98 H fl 80-97 MCH 33.7 H pg 27.5-33.5 MCHC 35.3 % 32.0-36.0 RDW 11.8 % 11.0-15.0 PLT 172 x 1000/mm 3 140-390 MPV 7.6 fl 7.5-11.5 NEUT % 40.1 % 38.0-80.0 LYMPH % 46.1 % 15.0-49.0 MONO % 12.9 % 0.0-13.0 EOS % 0.6 % 0.0-8.0 BASO % 0.3 % 0.0-2.0 NEUT, ABS 2085 cells/mm 3 1650-8000 LYMPH, ABS 2397 cells/mm 3 1000-3500 MONO, ABS 671 cells/mm 3 40-900 EOS, ABS 31 cells/mm 3 30-600 BASO, ABS 16 cells/mm 3 0-125 11

Red Blood Count and RBC Indices TEST RESULT UNITS REF RANGE WBC 5.2 x 1000/mm 3 3.9-11.1 RBC 3.81 L x 10 6 /mm 3 4.20-5.70 HGB 14.5 g/dl 13.2-16.9 HCT 41.2 % 38.5-49.0 MCV 98 H fl 80-97 MCH 33.7 H pg 27.5-33.5 MCHC 35.3 % 32.0-36.0 RDW 11.8 % 11.0-15.0 PLT 172 x 1000/mm 3 140-390 MPV 7.6 fl 7.5-11.5 NEUT % 40.1 % 38.0-80.0 LYMPH % 46.1 % 15.0-49.0 MONO % 12.9 % 0.0-13.0 EOS % 0.6 % 0.0-8.0 BASO % 0.3 % 0.0-2.0 NEUT, ABS 2085 cells/mm 3 1650-8000 LYMPH, ABS 2397 cells/mm 3 1000-3500 MONO, ABS 671 cells/mm 3 40-900 EOS, ABS 31 cells/mm 3 30-600 BASO, ABS 16 cells/mm 3 0-125 12

Measuring RBCs (and the Rule of Threes ) A x 3 = B x 3 = C A) RBC count is the number of red blood cells per microliter of whole blood. Measured in millions of RBCs per microliter of whole blood. Normal range for women: 4.0-5.2 x10 6 /mm 3 Normal range for men: 4.5-5.9 x10 6 /mm 3 B) Hemoglobin (HGB) measures the concentration of hemoglobin expressed as grams of hemoglobin per deciliter (100 ml) of whole blood. Normal range for women: 12-16 g/dl Normal range for men: 13.5-17.5 g/dl C) Hematocrit (HCT) is the percent of a volume of whole blood occupied by intact red blood cells. Measured in percent. Normal range for women: 36-46% Normal range for men: 41-53% 13

Red Blood Cell Indices Mean Corpuscular Volume (MCV) is the average size of red blood cells. Normal range: 80-100 fl Low = microcytic ( too small ) High = macrocytic ( too big ) Normal = normocytic ( just right ) Red Cell Distribution Width (RDW) measures the variability in the size of red blood cells. Normal range: 11.5-14.5% On a peripheral blood smear, high RDW is described as anisocytosis Mean Corpuscular Hemoglobin (MCH) is the amount of hemoglobin in an average red blood cell. Normal range: 26-34 pg/cell Mean Corpuscular Hemoglobin Concentration (MCHC) is the average concentration of hemoglobin in an average RBC. Normal range: 31-37 g/dl Hypochromic = too pale Normochromic = just right 14

Red Blood Cell Indices Mean Corpuscular Volume (MCV) is the average size of red blood cells. If anemia is present, MCV is a useful tool to guide further testing If anemia is not present, MCV is of little value: Low MCV without anemia suggests thalassemia minor (trait) High MCV without anemia can be caused by certain medications (Dilantin, Oral Contraceptives, Methotrexate) and is a soft marker of possible alcohol overuse Red Cell Distribution Width (RDW) measures the variability in the size of red blood cells. Not useful in the absence of anemia 16

Descriptive Terms Used on Peripheral Smears Anisocytosis: marked variation in RBC sizes (visual counterpart of increased RDW) Hypochromia or hypochromasia: RBCs are paler than normal because they contain less hemoglobin (visual counterpart of decreased MCH) Macrocytosis: increased number of large RBCs (visual counterpart of increased MCV) Microcytosis: increased number of small RBCs (visual counterpart of decreased MCV) Poikilocytosis: marked variation in the shape of RBCs 17

Red Blood Cell Morphology 18

Reticulocyte Count Reticulocytes are young red blood cells that were recently released from the bone marrow. Normally, reticulocytes comprise 0.5-2.5% of all red blood cells. Increased reticulocytes (reticulocytosis) is a normal response to blood loss or anemia. Since reticulocytes are larger, the MCV (and RDW) may be elevated. The combination of anemia with a low or normal reticulocyte count indicates that the bone marrow is unable to respond normally, either due to lack of essential ingredients (iron deficiency, vitamin B12 or folate deficiency), bone marrow disease, or chronic disease. 19

Mean Cell Volume (MCV) RBC volume (rather) is measured by MCV The Mean Cell Volume or MCV and RDW Microcytic Normocytic Macrocytic < 80 fl 80-100 fl > 100 fl

Anemia Workup - MCV MCV Microcytic Sideroblastic anemia Anemia of chronic disease Lead poisoning Thalassemias Iron deficiency Normocytic Hemolysis Anemia of chronic disease Marrow failure/disorders Macrocytic Megaloblastic anemias Liver disease/alcohol Metabolic disorders Marrow disorders (MDS) Drugs www.drsarma.in

Anemia Work-up 1 st test: Retic count 2 nd test: MCV 3 rd test: RDW 4 th test: Peripheral smear 22

Classification of Anemias Using MCV and RDW Low MCV (Microcytic) Normal MCV (Normocytic) High MCV (Macrocytic) Low RDW (low variability) Thalassemia minor Normal RDW Thalassemia minor Anemia of chronic disease Anemia of chronic disease Hereditary spherocytosis Aplastic anemia Myelodysplastic syndrome High RDW (high variability) Iron deficiency Early deficiency of iron, B12, or folate Sickle cell anemia B12 or folate deficiency Hemolytic anemia 23

Anemia Low RBCs three ways to get there: too little production, increased destruction, or bleeding Low production Aplastic anemia (no production) Iron deficiency anemia Low erythropoietin (kidney disease) or decreased response to erythropoietin (iron deficiency, B12 deficiency, folic acid deficiency, chronic disease) Increased destruction Hemolytic anemia Spherocytosis Hemoglobinopathies (sickle cell) Hypersplenism (an overactive spleen which destroys formed elements prematurely) Bleeding Chronic Acute 24

Anemia Regardless of the type of anemia, once the level of hemoglobin/hematocrit gets sufficiently low, mortality may result Mortality Concerns Severe anemia may cause a critical lack of oxygen to the brain or heart Less severe anemia may still worsen chronic heart or lung conditions For Financial Professional Use Only 25

Iron deficiency/hemorrhagic anemia The oxygen-carrying protein heme contains iron, and gives blood its red color If iron levels are low, RBCs cannot be produced fast enough to match losses Iron-deficiency anemia is the most common form of anemia world-wide May be due to poor intake of iron or chronic loss of blood Blood is lost most commonly from the GI tract Possible causes: Stomach ulcers Colon cancer Vascular malformations in the GI tract Endometrial cancer Recent surgery or trauma Multiple blood donations Can be treated with iron supplements, blood transfusion, and/or eradication of the cause 26

Iron Deficiency Anemia Lab Findings Serum Iron LOW (< 60 micrograms/dl) Total Iron Binding Capacity (TIBC) HIGH ( > 360 micrograms/dl) Serum Ferritin LOW (< 20 nanograms/ml) Can be falsely normal in inflammatory states

Treatment of Iron Deficiency Anemia Oral iron salts Ferrous sulfate 325 mg po Q Day Side effects: constipation, black stools, positive hemmoccult test Vitamin C can facilitate iron absorption.

Hemoglobin Disorders These are various, largely genetic, disorders which affect hemoglobin Those which affect globin: Thalassemias Sickle cell and related disorders Those which affect heme: Porphyrias (rare) 30

Thalassemias Inherited disorders of the globin gene leading to low levels of alpha or beta globin chains Fatalities are unusual in all but the most severe forms Alpha Thalassemia: Hemoglobin H disease Alpha Thalassemia Trait Beta Thalassemia: Beta Thalassemia major Beta Thalassemia intermedia Beta Thalassemia minor (trait) Prevalence as high as 10% in Mediterranean, African, and Southeast Asian populations Typically mild anemia with marked microcytosis and target cells Can coexist with other hemoglobin abnormalities, with increased severity of the anemia and increased mortality concern, but true beta-thalassemia minor (trait) has no excess mortality concerns 31

Sickle Cell Disease Forms of hemoglobin: A adult normal hemoglobin S sickle cell hemoglobin C mutant hemoglobin, but with a different result Since each person has 2 genes, several combinations are possible SA Sickle trait, may have minimal sickle cell formation, but otherwise is basically a carrier SS affected with sickle cell disease can be mild to severe SC similar to SS but milder CC - similar to SS but quite a bit milder Sickle Cell Disease: When oxygen level is low, RBCs assume a sickle shape, and block capillaries, leading to severe bone pain, and infarctions in various organs Death can occur from infection, severe anemia, stroke, or heart attack 33

Autoimmune Hemolytic Anemia A condition where the immune system attacks and destroys red blood cells Two forms: Warm antibody Cold agglutinin disease Both cause: Normocytic anemia with antibodies against red blood cells (positive Coomb s test) Mortality concerns Severity of anemia (hemolytic crises may be fatal) Presence of an underlying cause (such as infection or rheumatologic disease like rheumatoid arthritis) Need for side-effect prone treatments such as steroids, immunosuppressants, or frequent blood transfusions 35

Lab Analysis in Hemolytic Anemia Increased indirect bilirubin Increased LDH Increased reticulocyte count > 3% is sign of increased reticulocyte production, suggestive of hemolysis Reduced or absent haptoglobin < 25 mg /dl suggests hemolysis Haptoglobin binds to free hemoglobin released after hemolysis

Hereditary Spherocytosis/Elliptocytosis A disorder of red cell shape where the misshapen cells are taken up by the spleen and destroyed prematurely Leads to anemia and splenomegaly Splenectomy will normalize the blood count and resolve the anemia, but the cells will still be misshapen Mortality Concerns: Severity of hemolysis or anemia Overwhelming infection after splenectomy 38

Anemia of Chronic Disease Also known as anemia of chronic inflammation May occur in association with a wide variety of chronic diseases, especially inflammatory conditions, chronic infection, liver disease, congestive heart failure, diabetes, and cancer Anemia is usually mild (Hgb 9-12, Hct 27-35), but can be lower in about 20% of cases MCV is usually normal or low normal Platelets may be elevated, especially if inflammation is prominent 41

Megaloblastic Anemia A diverse group of anemias, all of which show elevated MCV on the blood count May be caused by: Vitamin deficiency (usually B12 or folic acid) Alcohol intake (moderately high levels) Leukemia (WBCs would be abnormal, too) Certain medications Pernicious anemia vitamin B12 deficiency caused by an inability to absorb the vitamin from the GI tract. May cause neuropathy, weakness, or even psychiatric problems Usually treated by Determining and eliminating the cause Correcting the vitamin deficiency (if any) May require injections of vitamin B12 if the cause is pernicious anemia Mortality concerns determined by the underlying cause and severity of anemia 42

Anemia due to B12 Deficiency Macrocytic anemia Lab Values Cobalamin level < 200 pg/ml Elevated serum methylmalonic acid Elevated serum homocysteine Vitamin B12 is needed for DNA synthesis Binds to intrinsic factor in the small bowel in order to be absorbed Pernicious anemia: antibodies to intrinsic factor Diagnosed by checking antibody levels (rather than Schilling test) Deficiency can result in neuropsychiatric symptoms Spastic ataxia, psychosis, loss of vibratory sense, dementia Frequently not reversible with cobalamin replacement Smear shows macrocytosis with hypersegmentation of polymorphonuclear cells, with possible basophilic stippling

Treatment of Vitamin B12 or FolateDeficiency Vitamin B12 1000 micrograms IM/SQ monthly -OR- Vitamin B12 1000-2000 micrograms po daily Folate 1 to 5 mg po daily Vitamin B12 deficiency must be excluded in folate-deficient patients, because supplemental folate can improve the anemia of Vitamin B12 deficiency but not the neurologic sequelae

Vitamin B12 Deficiency Versus Folate Deficiency Vitamin B 12 Deficiency MCV > 100 > 100 Folate Deficiency Smear Macrocytosis with hypersegmented neutrophils Macrocytosis with hypersegmented neutrophils Pernicious anemia Yes NO Homocystine Elevated Elevated Methylmalonic Acid Elevated NORMAL

White Blood Count with Differential TEST RESULT UNITS REF RANGE WBC 5.2 x 1000/mm 3 3.9-11.1 RBC 3.81 L x 10 6 /mm 3 4.20-5.70 HGB 14.5 g/dl 13.2-16.9 HCT 41.2 % 38.5-49.0 MCV 98 H fl 80-97 MCH 33.7 H pg 27.5-33.5 MCHC 35.3 % 32.0-36.0 RDW 11.8 % 11.0-15.0 PLT 172 x 1000/mm 3 140-390 MPV 7.6 fl 7.5-11.5 NEUT % 40.1 % 38.0-80.0 LYMPH % 46.1 % 15.0-49.0 MONO % 12.9 % 0.0-13.0 EOS % 0.6 % 0.0-8.0 BASO % 0.3 % 0.0-2.0 NEUT, ABS 2085 cells/mm 3 1650-8000 LYMPH, ABS 2397 cells/mm 3 1000-3500 MONO, ABS 671 cells/mm 3 40-900 EOS, ABS 31 cells/mm 3 30-600 BASO, ABS 16 cells/mm 3 0-125 47

Absolute Neutrophil Count TEST RESULT UNITS REF RANGE WBC 5.2 x 1000/mm 3 3.9-11.1 RBC 3.81 L x 10 6 /mm 3 4.20-5.70 HGB 14.5 g/dl 13.2-16.9 HCT 41.2 % 38.5-49.0 MCV 98 H fl 80-97 MCH 33.7 H pg 27.5-33.5 MCHC 35.3 % 32.0-36.0 RDW 11.8 % 11.0-15.0 PLT 172 x 1000/mm 3 140-390 MPV 7.6 fl 7.5-11.5 NEUT % 40.1 % 38.0-80.0 LYMPH % 46.1 % 15.0-49.0 MONO % 12.9 % 0.0-13.0 EOS % 0.6 % 0.0-8.0 BASO % 0.3 % 0.0-2.0 NEUT, ABS 2085 cells/mm 3 1650-8000 LYMPH, ABS 2397 cells/mm 3 1000-3500 MONO, ABS 671 cells/mm 3 40-900 EOS, ABS 31 cells/mm 3 30-600 BASO, ABS 16 cells/mm 3 0-125 5.2 x 1000 = 5200 5200 x.401 = 2085 48

White Blood Cell Count If the total white blood count (WBC) is normal, the red cells and platelets are normal, and the patient is otherwise healthy, don t worry about the differential white blood count, especially if the absolute counts are normal. The percentage of neutrophils, lymphocytes, etc. often fluctuate in response to an infection; this is not a big concern. 49

Types of White Blood Cells - What s the Diff? Neutrophils also called a variety of other names on CBC reports, including: polys PMNs segs bands or stabs (immature neutrophils indicate acute infection) Lymphocytes Monocytes Eosinophils Basophils 50

WBC Differential: Neutrophils Possible Causes of Neutrophilia: Possible Causes of Neutropenia: Common: bacterial infections, inflammatory disorders, stress, certain drugs (especially Prednisone), pregnancy Rare: leukemias Common: chronic benign neutropenia (some forms are familial), chemotherapy Uncommon: systemic lupus erythematosus, immunodeficiency states 51

Leukocytosis An elevated WBC count is termed leukocytosis Normal level is 4,400 to 10,000 WBC per mm 3 This can result from many causes, principally infections, inflammatory disorders, and medications Cancer and myeloproliferative disorders can also cause high, sometimes extremely high, WBC counts Treatment is aimed at the underlying cause Death may result from the underlying cause such as severe infection or cancer (leukemia) 52

Neutropenia The relative lack of neutrophils (the most common type of WBC, also called granulocytes) Normally humans have at least 1500 neutrophils/mm 3 Severe neutropenia (almost no neutrophils) is agranulocytosis May be cause by gene defects, various drugs, or medical conditions Treatment is usually aimed at the underlying cause Some may require antibiotics to prevent infection or medications to stimulate production of neutrophils Death may occur due to overwhelming infection Chronic Benign Neutropenia: Overall risk of infection is low, usually asymptomatic Two forms familial and non-familial Key feature is the absence of significant infections and stability over time 53

WBC Differential: Lymphocytes Possible Causes of Lymphocytosis: Possible Causes of Lymphopenia: Common: viral infections Uncommon: inflammatory bowel disease Rare: chronic lymphocytic leukemia, vasculitis Uncommon: systemic lupus erythematosus, immunodeficiency states Rare: aplastic anemia, Hodgkin s disease 54

WBC Differential: Monocytes Possible Causes of Monocytosis: Common: recovery phase after infections Uncommon: certain infections (TB, malaria), inflammatory bowel disease Rare: myeloproliferative disorders including myeloid metaplasia, polycythemia vera, certain forms of leukemia and lymphoma Decreased Levels: Infection (HPV), certain skin disorders, chemotherapy, bone marrow disorder 55

WBC Differential: Eosinophils Possible Causes of Eosinophilia: Decreased Levels: Common: allergic disorders (including drug reactions) Uncommon: parasite infection, lupus, rheumatoid arthritis Rare: hypereosinophilic syndrome, diffuse skin diseases, some forms of leukemia and lymphoma, Löffler s endocarditis Alcohol, Cushing syndrome, sepsis, treatment with steroids 56

WBC Differential: Basophils Possible Causes of Basophilia: Decreased Levels: Rare: leukemias, myeloid metaplasia, Hodgkin s disease Hyperthyroidism, infections, allergic reaction 57

Platelet Count TEST RESULT UNITS REF RANGE WBC 5.2 x 1000/mm 3 3.9-11.1 RBC 3.81 L x 10 6 /mm 3 4.20-5.70 HGB 14.5 g/dl 13.2-16.9 HCT 41.2 % 38.5-49.0 MCV 98 H fl 80-97 MCH 33.7 H pg 27.5-33.5 MCHC 35.3 % 32.0-36.0 RDW 11.8 % 11.0-15.0 PLT 172 x 1000/mm 3 140-390 MPV 7.6 fl 7.5-11.5 NEUT % 40.1 % 38.0-80.0 LYMPH % 46.1 % 15.0-49.0 MONO % 12.9 % 0.0-13.0 EOS % 0.6 % 0.0-8.0 BASO % 0.3 % 0.0-2.0 NEUT, ABS 2085 cells/mm 3 1650-8000 LYMPH, ABS 2397 cells/mm 3 1000-3500 MONO, ABS 671 cells/mm 3 40-900 EOS, ABS 31 cells/mm 3 30-600 BASO, ABS 16 cells/mm 3 0-125 58

Mean Platelet Volume (MPV) Young platelets, recently released from the bone marrow, are typically slightly larger Often elevated in immune or idiopathic thrombocytopenic purpura (ITP) In an individual with low platelet count (thrombocytopenia): Increased MPV indicates normal bone marrow response Decreased or low normal MPV may indicate impaired bone marrow response 59

Selected Causes of Abnormal Platelet Counts Possible Causes of Thrombocytosis: Common: Reactive thrombocytosis related to acute trauma, surgery, blood loss, iron deficiency, chronic infections (osteomyelitis), inflammatory diseases including rheumatoid arthritis and ulcerative colitis, splenectomy Uncommon: polycythemia vera, essential thrombocytosis (plt count over 600,000), some cancers Possible Causes of Thrombocytopenia: Common: spurious lab result caused by platelet clumping, idiopathic or immune-mediated thrombocytopenia (ITP), medications, viral infection Uncommon: hemolytic-uremic syndrome, leukemia, sepsis, hypersplenism 60

Idiopathic Thrombocytopenic Purpura (ITP) Although there are many causes of low platelet counts, including medications, infections, and hypersplenism, the term ITP is reserved for the autoimmune destruction of platelets; immune thrombocytopenic purpura Immune system attacks and destroys platelets faster than they can be produced; red rash (purpura) and bleeding Some cases will remit and never recur If more severe, can usually be cured with splenectomy, but some will still have low platelets after splenectomy Prognosis depends on the current platelet count and the mode of treatment Mortality Concerns Fatalities may occur due to severe bleeding or due to overwhelming infection after splenectomy 61

Bone Marrow Disorders All formed elements of the blood are made primarily in the bone marrow There is particular concern if all 3 cell lines are abnormal (RBCs, WBCs, and platelets) This suggests the possibility of diseases involving the bone marrow, and many of these diseases have very significant mortality concerns Aplastic anemia: diminished-absent precursor cells (pancytopenia) Myeloproliferative disorders: bone marrow cells grow too rapidly with over-production of blood cells and/or platelets If this acceleration continues it may transform into cancer (usually leukemia) Myeloproliferative disorders include: Essential thrombocytosis Polycythemia vera Myelofibrosis (possible pancytopenia) Chronic myelogenous leukemia Myelodysplastic syndromes: ineffective blood cell production (pancytopenia) 62

Aplastic Anemia A disorder where the bone marrow stops producing blood cells (pancytopenia - all lines RBC, WBC and platelets) Can be congenital (Fanconi anemia) or acquired Acquired form brought on by certain infections or drugs Prognosis depends on severity most cases pose a very high mortality risk unless spontaneously remitted long ago Mortality Concerns: The aplastic crisis may result in death due to infection, bleeding, or severe anemia Complications of bone marrow transplant (graft vs. host disease) 63

Essential Thrombocytosis (ET) Also called essential thrombocythemia Megakaryocytes in the bone marrow produce too many platelets, which may be misshapen or may function poorly They may become so numerous as to cause clotting (risk of stroke) Platelet count at least 600k up to 1.5 million per mm 3 Prognosis depends on frequency of bleeding/clotting complications 64

Polycythemia (rubra) vera Red cell progenitors start to lose control over their growth Can be treated with phlebotomy (removing blood) or marrow-suppressing medications Mortality Concerns Fatalities can occur due to clotting or due to malignant transformation 65

Myelofibrosis A condition where the bone marrow becomes fibrotic (scarred) Can cause low counts of all cell lines (pancytopenia) Spleen and liver may become enlarged as they begin to serve as back-up producers of formed elements (extramedullary hematopoesis) Mortality Concerns: decreasing counts of all formed elements may lead to death from infection, severe anemia, or bleeding 66

Chronic Myelogenous Leukemia Cancer of the white blood cells Increased and unregulated growth of myeloid cells in the bone marrow Philadelphia chromosome (bcr-abl t(9;22)) Treated with Tyrosine Kinase Inhibitors 67

Myelodysplasia A bone marrow disorder of older individuals where there is ineffective production of red blood cells, eventually may also involve the WBC and platelet lines (pancytopenia) Can be a precursor to leukemia Several subtypes, e.g., refractory anemia (RA) or refractory anemia with ringed sideroblasts (RARS) Treatment is supportive transfusions and antibiotics Prognosis is generally poor Mortality concerns: malignant transformation, severe anemia, infection, or bleeding 68

The Good, The Bad, and The Ugly Common Abnormal but Benign CBCs (CBC may be accepted without repeat CBC or clinical evaluation) Mild iron deficiency anemia in pre-menopausal women Beta thalassemia minor Chronic benign neutropenia Reactive thrombocytosis Abnormal / Worrisome CBCs (may require additional follow-up or clinical evaluation) Iron deficiency anemia in men or post-menopausal women Mild or moderate anemia, not fully evaluated Unexplained thrombocytopenia or thrombocytosis Mild abnormalities involving all 3 cell lines (red cells, white cells, and platelets), mild pancytopenia Markedly Abnormal CBCs Leukemia Severe anemia Severe leukopenia or severe pancytopenia 69