Hematology - Full Spreadsheet Info Only Flashcards
Iron Deficiency Anemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Inadequate supply of iron for synthesis of hemoglobin
Etiology Iron absorption >1-1.5 mg/day (after absorption) due to:
- GI tract bleeding (acute or chronic)
- Chronic asprin or NSAID use
- Low iron intake
- Increased Fe demands
- Pregnancy/lactation
- Heavy menstrual bleeding
**Pathophysiology **Heme molecule cannot bind oxygen without sufficient oxygen
Symptoms
- Brittle nails
- Koilonychia (spoon shaped nails)
- Pale conjunctiva
- Smooth tongue (typical of all malnutrition anemias)
- Tachycardia
Signs
- Fatigue
- Weakness
- Palpitations
- SOB
- Pica
Differential Dx c differentiating factors
- Anemia of chronic disease
- Normal ferritin levels
- Low Serum Fe
- Thallesemia
- Sig. low MCV
- Normal or inc. RBC
- Sideroblastic anemia
- Inc. serum Fe
- Inc. % saturation
Diagnostic Tests Listed in order of appearance of sig finding c disease progression
- CBC – microcytic hypochromic RBC in late anemia progression
- Serum ferritin – <12 mcg/dl is highly reliable (labs will provide norm. ranges)
- Serum Fe
- Total Iron Binding Capacity (TIBC)
- % Saturation (of Fe in proteins)
Medical Tx Dependent on cause. Ultimately tx underlying reason
- Ferrous sulfate 325 mg pot id
- Expect normal hematocrit within 3 weeks
- Expect baseline within 2 mo
- Continue 6 mo to restore Fe stores in liver and heart
- Parenteral Fe (only if oral fails)
Surgical Tx None
Emergency Tx None
Pt Education/Prevention Once levels are returned to normal, practice etiology-specific practices to prevent recurrence
Misc.
- Most common cause of anemia worldwide (20% of white female Americans)
- Parenteral Fe must be delivered in hospital due to high risk of anaphylaxis
Anemia of Chronic Disease
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Anemia from inflammatory disease à impaired marrow response to EPO
Etiology Associated c chronic diseases that chronically inflame
- Rheumatoid arthritis
- Chronic infection
- Malignancy
- Chronic renal disease
Pathophysiology
Disruption originating from macrophages (still in research)
- Macrophage release of cytokines (interleukins)
- IL-6, IL-1β
- Increased liver production of hepcidin
- Hepcidin decreases iron absorption form GI epithelium
- Hepcidin decreases recycled iron export from macrophages due to inhibited ferroportin
- TNF-α, IL-1β
- Inappropriate EPO production in kidney
- Reduced erythropoiesis in BM
- IL-6, IL-1β
Bone marrow dysfunction due to various causes
- T-cell mediated autoimmune suppression of hematopoiesis (most common)
- Radiation therapy
- Chemotherapy
- Toxins
Symptoms
- Fatigue
- Weakness
- Symptoms consistent c underlying cause
Signs
- Pallor
- Signs consistent c underlying cause
Differential Dx
- Iron deficiency anemia
- Thallesemia
- Sideroblastic anemia
Diagnostic Tests Order varies dependent on history
- CBC – normochromic normocytic anemia or microcytic, hypochromic anemia (90% of time microcytic)
- Serum Fe – decreased, iron sequestered in macrophages
- TIBC – decreased
- Serum ferritin – normal or increased, adequate iron in body but hepcidin prevents uptake
Medical Tx Treat the underlying disease and give EPO injections
- CRI – hemodialysis + EPO
- Malignancy – chemo, radiation, EPO
- Inflammation (RA) – NSAIDs, DMARDs, EPO
Surgical Tx None
Emergency Tx None
Pt Education/Prevention Understanding of underlying disease
Misc.
- Chronic renal insufficiency (CRI) chronic anemia comorbid c iron and folate deficient anemias due to loss of RBCs
- Differentiation between this and iron deficiency is often on boards
Thalasemias, group of diseases
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Hereditary disorders characterized by reduction in synthesis of globin chains (α or β)
Etiology
- α thal – HBA1 and HBA 2 gene deletion causing reduced α-globin chain synthesis. Severity depends on how many present alleles out of 4
- β thal – gene mutation terminating DNA chain responsible for β-globin prdctn. May be homozygous (major) or homozygous (minor)
Pathophysiology Reduced globin chain synthesis → reduced hemoglobin synthesis → hypochromatic mircocytic anemia
Symptoms Typical anemia symptoms c varying severity depending on etiology
Signs
- α Thalasemia, severity depends on number of missing alleles
- 4 alleles – normal hbg
- 3 alleles – silent carrier, asymptomatic
- 2 alleles – “trait” exhibits mild microcytic anemia symptoms
- 1 allele – “Hbg H Disease” variable severity splenomegaly and pallor
- 0 alleles – “hydrops fetalis” stillborn fetus
- β Thalasemia
- Major – symptom development upon unsuccessful Hbg F à Hbg A conversion
- Growth failure
- Bone deformities
- Hepatosplenomegaly
- Jaundice
- Minor –clinically insignificant microcytic anemia (often discovered in routine physical
Differential Dx c differentiating factors
Iron deficient anemia, due to microcytic hypochromatic cells
- Higher MCV
- Abnormal Fe studies
Diagnostic Tests
- CBC –
-
Microcytosis out of proportion to degree of anemia, RBCs also hypochromatic
- MCV 55-70 fl
- Poikilocytosis – abnml RBC shape
- Hgb between 3 and 6 gm/dL
-
Microcytosis out of proportion to degree of anemia, RBCs also hypochromatic
- Serum Fe – normal
- Hemoglobin electrophoresis – definitive dx
Medical Tx
- Mild (α thal trait or β thal minor) – no tx
- Hgb disease – folic acid supplementation
- β Thalasemia major
- Chelation therapy
- Transfusions
- Folate supplementation
Surgical Tx Stem cell/bone marrow transplantation for β Thalasemia major
Emergency Tx None
Pt Education/Prevention
- Highly inheritable, take a thorough hx
-
Avg patient populations
- α thal – SE Asian
- β thal – Mediterranean (Greek, Sicilian)
Misc. Pts usually live to 30’s until overcome c heart failure
Sideroblastic Anemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Genetic or acquired defective heme synthesis
Etiology
- Lead
- EtOH abuse
- Myelodysplasia (umbrella term)
Pathophysiology Reduced Hgb synthesis due to inability to incorporate heme into protoporhyn. Results in iron accumulation in mitochondria.
Symptoms Consistent c anemia, no identifying s/sx
Signs Consistent c anemia, no identifying s/sx
Differential Dx
- Iron deficiency anemia
- Thallesemia
- Anemia of chronic disease
Diagnostic Tests
- CBC
- Normochromatic, micro AND macrocytic anemia
- Basophilic stippling of RBC – rRNA remnants from impaired hgb synth
- Serum Fe – increased
-
Bone marrow biopsy – diagnostic
- Erythroid hyperplasia
- Ringed sideroblasts (iron ring within nucleated RBC)
Medical Tx Manage offending agent
Surgical Tx None
Emergency Tx None
Pt Education/Prevention None
Misc. None
Vitamin B12 (Cobalamine) Deficiency
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Inadequate supply of vitamin B12 for erythrocyte DNA synthesis
Etiology
- Pernicious anemia (autoimmunity directed at parietal cells)
- Strict vegan diet (decreased B12 intake)
- Gastric surgery (bypass parietal cells)
- Crohn’s Disease
Pathophysiology Inadequate B12 absorption due to
- Lack/dysfunction/bypass of terminal ilium parietal cells responsible for B12 absorption
- Lack of intrinsic factor (IF) in parietal cells
- Lack of B12 intake
Leads to impaired erythroblast DNA assembly, despite normal cytoplasm proliferation. Results in macrocytic cells c poor oxygen carrying capacity.
Symptoms Typical anemia symptoms
Signs
- Glossitis c burning sensation
- Neuro dysfunction
- Stocking glove parathesia** **
- Loss of sensation in position, fine touch, vibration
- Clumsiness and ataxia
Differential Dx Folic Acid Deficiency Anemia
Diagnostic Tests
- CBC
- Severe anemia
- MCV >100 (macrocytic)
- Hypersegmented neutrophils
- Anisocytosis
- Poikilocytosis
- Howell Jolly bodies
- Vitamin B12 assay = decreased
- Folate assay = normal
- Shilling test – antiquated
Medical Tx
- Vitamin B12 supplimentation
- First month = weekly IM injections
- Remainder of life = monthly IM injections, 1000 mcg
- Oral cobalamin, 0.5-2 mg/day
- Only if pt is lacking B12 intake due to necessity of functional parietal cells to absorb
- OTC SQ tablet, not as effective
- Nightly nasal spray, not as effective and expensive
Surgical Tx None
Emergency Tx None
Pt Education/Prevention
- Hemostalogic correction usually occurs within 6 weeks
- Neurologic improvement may take up to 18 months, if at all
Misc.
- Patients may try to self-treat the glossitis c oral rinses. Since this is a neuro sign the rinces will not work but the bacteria in the mouth will be eliminated, often resulting in Candida
- Cells may never appear macrocytic. In suspicion of B12 anemia, the most reliable test is the B12 assay
Folic Acid Deficiency
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Inadequate supply of vitamin B12 for erythrocyte DNA synthesis
Etiology
-
Poor dietary intake
- Alcoholics
- Anorexia (psychological or secondary to comorbidity like cancer)
- Diet low in citrus fruits and dark leafy greens
- Malabsorption (celiac, Crohn’s)
- Pregnancy
- Hemolytic anemia
Pathophysiology Inadequate folic acid absorption leads to impaired erythroblast DNA assembly, despite normal cytoplasm proliferation. Results in macrocytic cells c poor oxygen carrying capacity.
Symptoms Typical to anemia
Signs
- Glossitis
- Vague GI complaints
- Anorexia
- Nausea
- Abd Pn
- Constipation/diarrhea
- No neurologic s/sx (differentiating factor from B12)
Differential Dx B12 Deficiency
Diagnostic Tests
- CBC
- Severe anemia
- MCV >100 (macrocytic)
- Macro-ovalocytes
- Hypersegmented neutrophils
- Howell Jolly bodies
- Vitamin B12 assay = normal
- Folate assay = decreased
Medical Tx
- Folic acid 1 mg po daily
- Avoid alcohol
- Avoid folic acid antagonists, or supplement if avoidance is unreasonable
- Methotrexate
- Trimethoprim sulfate (antibiotic)
- Phenytoin (dilantin)
Surgical Tx None
Emergency Tx None
Pt Education/Prevention Pregnant women c folic acid anemia may lead to fetuses c neural tube defects. Recommendation: start folic acid tx 3 mo before consuming
Misc. None
Hereditary Spherocytosis
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Genetic hemolytic anemia due to fragile spherical erythrocytes
Etiology Autosomal dominant disorder
Pathophysiology
- RBCs produce deficient number of membrane PRO
- RBC form c decreased surface:volume ratio and fragile spherical shape
- Decreased flexibility causes hemolysis when passing through spleen
Symptoms
- RUQ Pain
- Family history
Signs
- Jaundice noted by scleral icterus
- Gallstones c predominating bilirubin crystal composition
- Palpable spleen
Differential Dx Other hemolytic anemias
- G6PD deficiency
- Sickle cell disease
- Autoimmune hemolytic anemia
- Microangiopathic hemolytic anemia
- Aplastic anemia
Diagnostic Tests
- CBC
- Variable anemia c aplastic crises concurrent c BM stress
- Dimorphic population including spherocytes
- Microcytosis c increased MCHC (>36 g/dl)
- Reticulocyte count increased
- Indirect bilirubin increased
- Osmotic fragility test – positive
Medical Tx Folic acid 1 mg/d
Surgical Tx Splenectomy
Emergency Tx None
Pt Education/Prevention Genetic counseling for future children
Misc. None
G6PD Deficiency
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Episodic hemolytic anemia due to glucose 6 phosphate dehydrogenase deficiency
Etiology X-linked recessive disorder, common among African black men who encounter an oxidative stress:
- Infection (viral or bacterial)
- Hypoxia
- Drugs
- Quinidine
- Sulfa drugs (trimethoprim sulfa)
- Nitofurantoin (macrodantin)
- Asprin
Pathophysiology
- RBC lose G6PD as they age
- Lack of G6PD decreases RBC’s ability to deal c oxidative stress (crisis)
- Oxidized hgb denatures to form Heinz bodies
- Heinz bodies formation damages the membrane
- Damaged RBCs removed by spleen
Symptoms Asymptomatic until oxidative stress. Stress induces SOB and organ specific hypoxia complaints
Signs Without signs until oxidative stress
- Hypoxia
- SOB
- Cyanosis
- Jaundice noted by scleral icterus
- Organ specific s/sx related to hypoxia
Differential Dx Other hemolytic anemias
- Hereditary spherocytosis
- Sickle cell disease
- Autoimmune hemolytic anemia
- Microangiopathic hemolytic anemia
- Aplastic anemia
Diagnostic Tests
- CBC and blood smear
- Heinz bodies
- Bite cells
- Reticulocyte count increased
- Indirect bilirubin increased
- G6PD assay
- During crisis – non revealing
- Weeks after crisis – deficient
Medical Tx Remove hypoxia source
Surgical Tx None
Emergency Tx Only if hypoxia is severe
Pt Education/Prevention Educate on how to avoid triggors
**Misc. **None
Sickle Cell Disease/Trait
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Chronic illness resulting in anemia from abnormal hemoglobin
Etiology Autosomal recessive genetic disorder where Valine substitutes for glutamine on beta chain. Most common in Africans (1/400 are homozygous)
Hemoglobinopathy Variations:
- Hgb SS – sickle cell disease
- Hgb AS – sickle cell trait
Pathophysiology
- Infants are born normally c predominating Hgb F levels
- S/sx appear as Hgb f is replaced by abnormal Hgb S
- Underlying anemia due to splenic attempt to clear abnormal cells
- Crises – Upon oxidative stress Hgb S crystallizes and sickles RBC shape. These get caught throughout system causing pain and potential organ swelling, dysfunction, and infarction
Symptoms Asymptomatic unless in crisis. Crisis symptoms may occur in varying frequency/duration and are characterized as acute pain due to vaso-occlusive episodes:
- Hypoxia
- Infarction
- Dehydration
Common pain sites
- Spine
- Long bones
- Chest
Signs Asymptomatic unless in crisis. Crisis signs may occur in varying frequency/duration and are characterized as
- Jaundice
- Pigment gallstones
- Splenohepatomegaly (esp in peds)
- Blood pooling in extremities – edema/ecchymosis/ulcer
- +/- low grade fever
- Vaso-occlusive crisis may lead to
- CVA
- Priapism
- MI
- PE
- Ischemic necrosis
- Liver
- Bone – Staph related osteomyelitis
Permanent anatomic changes due to repeated crises
- Poorly healing ulcers overlying distal tibia
- Scull “towering” and “bosses”
Associated Mortality
- Infections
- Pulmonary emboli
- Occlusion of vital vessel
- Renal failure
Crisis stimulation for sickle cell trait is much more extreme – ie rigorous exercise at high altitude
Differential Dx Other hemolytic anemias
- Hereditary spherocytosis
- G6PD deficiency
- Autoimmune hemolytic anemia
- Microangiopathic hemolytic anemia
- Aplastic anemia
Diagnostic Tests
- CBC
- Hct increased
- WBC count increased – 12,000-15,000/mcl
- Nucleated RBC’s
- Peripheral blood smear
- 5-50% RBC are sickled
- Howell Jolly bodies
- Target cells
- Reticulocyte count increased
- Bilirubin assays – both total and indirect increased
- Hbg electrophoresis –confirmatory test
- Sickle Cell Disease
- Hgb S 85-98%
- Hgb F 5-15%
- Hgb A2 1-3%
- Hgb A 0%
- Sickle Cell Trait
- Hgb A 60%
- Hgb S 40%
- Sickle Cell Disease
Medical Tx
- Folic acid supplementation
- Pneumococcal vaccine
- Transfusions as needed
- Hydroxyurea to inc. Hgb F levels
Surgical Tx Allogenic hematopoietic stem cell transplant from sibling donor
Emergency Tx Crisis Tx
- Vigorous oral or IV hydration
- Analgesics (morphine)
- Narcotics
- Treat precipitating factor – Oxygenate for hypoxia
- Blood transfusions
- If Hgb < 5mg/dl
- Cardiac failure
Pt Education/Prevention
- Life expectancy for disease = 40-50 years
- Behavior change to avoid crises if possible
**Misc. **None
Microangiopathic Hemolytic Anemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Group of disorders characterized by RBC fragmentation
Etiology
- Disseminated intravascular coagulopathy
- Thrombotic thrombocytic purpura
- Hemolytic uremic syndrome
- Malfunctioning cardiac valve prosthesis
- Malaria
-
Drugs
- Nitrofurantoin
- Sulfonamide
- Chloramphenicol
- Primaquine
Pathophysiology Variable source of fragmentation; most popular is RBC interaction c abnormal intravascular fibrin strands. RBC fragments are not useful and are disposed of in spleen
Symptoms Depends on underlying cause. May resemble iron deficiency in heart valve-related cases
Signs Depends on underlying cause. May resemble iron deficiency in heart valve-related cases
Differential Dx
- Iron deficiency anemia
- Other hemolytic anemias
- Hereditary spherocytosis
- G6PD deficiency
- Sickle cell disease
- Autoimmune hemolytic anemia
- Aplastic anemia
Diagnostic Tests
- Peripheral Smear indicates fragmented RBCs
- CBC may reveal decreased Hct and Hgb
Medical Tx Treat the underlying disorder
Surgical Tx None
Emergency Tx None
Pt Education/Prevention None
Misc. None
Aplastic Anemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Pancytopenia due to bone marrow failure
Etiology
- Idiopathic
- SLE (lupus) or any autoimmune disease
- Chemotherapy
- Radiation therapy
-
Toxins
- Benzenes (organic)
- Toluenes (organic)
- Insecticides (inorganic)
- Drugs
- Chloramphenicol
- Sulfonamides
- Phenytoin
- Carbamazepine
- Pregnancy (uncommon)
Pathophysiology Hematopoeitic stem cell injury/abnormal expression causes defects in all blood cell lines
Symptoms Anemia effects
- Fatigue
- Weakness
Signs
- Anemia effects
- Pallor
- Leukopenia effects
- Increased infections
- Thrombocytopenia effects
- Mucosal/skin bleeding
- Purpura
- Petechiae
Differential Dx
- Increased Blasts - Acute Leukemia
- Sepsis
- Normomarrow pancytopenia
- SLE (lupus)
- Hypersplenism
- Disseminated infection
Diagnostic Tests
- CBC
- Pancytopenia
- Blast cells decreased
- Reticulocyte count decreased
- Bone marrow biopsy hypocellular
Medical Tx
Refer to hematologist, who will do the following
- Blood Transfusion
- Platelet transfusion
- Antibiotics
- Cyclosporine and antithymocyte globulin
- Tx of choice for pts > 50 yo
Surgical Tx Allogenic bone marrow transplant if < 50 yo
Emergency Tx None
Pt Education/Prevention None
Misc. None
Acute Lymphocytic Leukemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Acute unrestricted growth of lymphocytes and their precursors
Etiology
- Most common pediatric leukemia (80%), years 3-7
- Adult, 20% of leukemias
- Family hx
- Ionizing radiation exposure
- Benzene
- Some alkylating agents (cyclophosphamide)
- Prognosis varies upon
- Age
- WBC
- Presence of Philadelphia chromosome
Pathophysiology Mutation to only the lymphocyte precursor. Produces excess and ineffective T, B, and NK cells. Excess cells “clog” the body and do not
Symptoms Reflective of dysfunctional lymphoid cells
- Fatigue
- Lethargy
- HA
- Bone pain
- Joint pain
Signs Reflective of dysfunctional lymphoid cells
- Fever
- Pallor
- Lymphadopathy
- Hematosplenomegaly
Differential Dx
- CLL
- Aplastic Anemia
- Bacterial Infection
- Sepsis
Diagnostic Tests
- CBC – high WBC count (lymphocytosis)
-
Bone marrow biopsy > 20% nucleated BM cells are lymphoblasts
- Confirmatory test
- CXR – mediastinal mass
- Genetic testing – Philadelphia chromosome sometimes present
- Unfavorable dx
Medical Tx
- Intensive chemotherapy
Surgical Tx None
Emergency Tx None
Pt Education/Prevention
- Prognosis – remission rate >70%
- Goal is to achieve high quality of life. If this is present without tx (especially in geriatric pts) then tx is not necessary
Misc. None
Acute Myelogenous Leukemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Acute unrestricted growth of myelocytes and their precursors, characterized by short duration of s/sx
Etiology
- Age – primary leukemia of people >60 yo
- Risk factors
- Family hx
- Ionizing radiation exposure
- Benzene
- Some alkylating agents (cyclophosphamide)
Pathophysiology Mutation to only the myelocyte precursor. Produces excess and ineffective granulocytes, monocytes, thrombocytes, and erythrocytes. Excess cells “clog” the body and do not
Symptoms Reflective of dysfunctional myeloid cells
- Bleeding
- GI
- Epistaxis
- Skin
- Menorrhagia
- Bacterial infections
- Joint pain
- Bone Pain
Signs Reflective of dysfunctional myeloid cells
- Pallor
- Gingival hyperplasia
- Purpura
- Petechiae
- Lymphadopathy
Differential Dx
- CML
- Aplastic Anemia
- Bacterial Infection
- Sepsis
Diagnostic Tests
- CBC – high WBC count (leukocytosis)
-
Bone marrow biopsy > 20% nucleated BM cells are myeloblasts
- Confirmatory test
- Auer Rods
- Peripheral smear contains myeloblasts
Medical Tx Chemotherapy
Surgical Tx Bone marro transplant
Emergency Tx None
Pt Education/Prevention
- Prognosis – c chemotherapy, complete remission in (depending on severity)
- 80-90% pts < 60 yo
- 50-60% pts > 60 yo
Misc. None
Chronic Myelogenous Leukemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Myeloproliferative disorder of extended duration
Etiology
- Young to middle aged adults (median age 55)
- Risk factors
- Family hx
- Ionizing radiation exposure
- Benzene
- Some alkylating agents (cyclophosphamide)
Pathophysiology Abnormal myeloid cell production c normal cell differentiation. Bone marrow function persists in early disease. Occurs in three phases
- Chronic
- Normal BM function
- Accelerated
- Impaired neutrophil differentiation
- WBC uncontrollable by chemo
- Acute (blast crisis)
- Myeloid blast proliferation
- Resembles AML
Symptoms
- Fatigue
- Weight loss
- Low grade fever
- Night sweats
- Abdo fullness
Signs
- Splenomegaly
- Sternal tenderness
Differential Dx
- AML
- CLL
- Bacterial Infection
- Sepsis
Diagnostic Tests
- CBC
- Increased WBC (leukocytosis > 150,000/mcl) c severe left shift
- Anemia – present in later stages
- Thrombocytosis – present in later stages
- Genetic testing
- Philadelphia chromosome (95% of the time)
- Bcr/abl gene*
Medical Tx Refer to hematologist, who will do the following
- First line – Imatinib
- Second line – Dasatinib
Surgical Tx Third line – BM transplant
Emergency Tx None
Pt Education/Prevention Imatinib often achieves remission x 8 years
Misc. Pts c unexplained leukocytosis < 150,000 can be referred to hematology/oncology for monitoring. They may start chemo at lower threshold
Chronic Lymphocytic Leukemia
(Definition, Etiology, Pathophysiology, Symptoms, Signs, Differential Dx, Dx Studies, Medical Tx, Surgical Tx, Emergency Tx, Pt Education/Prevention, Misc.)
Definition Clonal malignancy of B lymphocytes
Etiology Most prevalent leukemia
- Median age > 70 yo
- Risk factors
- Family hx
- Ionizing radiation exposure
- Benzene
- Some alkylating agents (cyclophosphamide)
Pathophysiology B lymphocyte clone will uncontrollably proliferate, creating ineffective B cells, thus ineffective plasma cells and antibodies. Note – B cell only, not entire lymphocyte line
Symptoms
- Fatigue
- Recurrent infections
Signs
- Splenomegaly
- Lymphadopathy
Differential Dx
- ALL
- Infection
- CML
- Sepsis
Diagnostic Tests
- CBC
- High WBC count (leukocytosis > 20,000/ml)
- +/- anemia
- +/- thrombocytosis
- peripheral lymphocytosis
- Peripheral smear reveals smudge cells
Medical Tx
- Early disease – observation
- Tx indications – S/sx that interfere c quality of life
- Progressive fatigue
- Symptomatic lymphadopathy, anemia, thrombocytopenia
- Chemotherapy
- Fludarabine
- Rituxamib (Retuxan)
Surgical Tx Allogenic stem cell transplant for pts who do not respond to chemo
Emergency Tx None
Pt Education/Prevention Mean survival time = 6 years. Choice to treat depends on quality of life and is completely up to the patient . Prognoses:
- Stages 0 or 1 = 10-15 years
- Stages 3 or 4 = 2 years
Misc. None