Hematologic Disorders Flashcards
examples of blood disorders
- hemochromatosis
- anemia
- leukocytosis
- leukopenia
- leukemia
- hodgkin’s
- non-hodgkins lymphoma
- polycythemia vera
- thrombocytosis
- thrombocytopenia
- sickle cell disease
- thalassemias
- hemophilias
- splenomegaly
- multiple myeloma
Hemochromatosis
- accum of excess iron in many organs
- especially liver, pancreas, skin
- leads to cell damage and functional insufficiency
Disorders of Iron Absorption
-Hemochromatosis
Early Symptoms of Hemocrhomatosis
- fatigue
- heart palpitations
- imporence
- loss menstrual periods
S/Sx Hemochromatosis
- severe weakness/fatigue
- joint pain/abdominal pain (2nd/3rd MCP)
- signs liver disease, diabetes, heart probs
- impotence, infertility, loss menstrual periods
- porphyria, hepatitis
Hemochromatosis Blood Tests
- elevated:
- -transferrin saturation >50%
- -serum ferritin >1000 ng/ml
- -transaminase
- -blood glucose
Later Symptoms of Hemochromatosis
- grey/bronze skin
- cirrhosis of liver
- liver cancer
- DM
- hypopituitarism
- decreased pit/gonadal function
- joint disease
- heart failure
- abdominal pain
- fatigue
- certain infections
Disorderd of Erythrocytes
-Anemias
Anemia
- reduction of total number of RBC in blood or decrease in quality/quantity of Hb
- affects O2 transport
Anemias result from:
- decreased RBC production
- increased RBC destruction
- combo
Anemia S/Sx
- pallor
- SOB
- palpitations
- lethargy
- easily fatigued
decreased prod of RBC–>
- altered Hb synthesis
- altered DNA synthesis
- bone marrow infiltration
- red cell aplasia (fail to develop)
- kidney disease (erythropoietin)
Anemia Pathophysiology:
- decreased/defective production of RBC
- increased RBC destruction
- Blood loss
cause of RBC destruction
-infection causing hemolysis
Diagnostic Tests of Anemia
- HCT/Hb
- size of RBC
- serum iron level-serum ferritin
- iron-binding capacity in blood
- blood factors needed to absorb vitamins
- CBC
Types of Anemia
- pernicious (lack B12)–macrocytic
- folate–macrocytic
- iron-deficiency
- sideroblastic
- hemolytic
- post-hemorrhagic
- anemia of chronic disease
Pernicious Anemia
- autoimmune response
- lack intrinsic factor for B12 absorption
- possible genetic link
Pernicious Anemia more common in
- northern europeans
- female>males
- genetic
Pernicious Anemia Sx
- weakness
- fatigue
- paresthesias (feet/fingers)
- difficulty walking
- loss appetite
- abdominal pain
- atrophic glossitis
- skin yellow
B12 needed for
-myelin in axons and SC
atrophic glossitis
- beefy red tongue
- swollen with no center fold
Pernicious Anemia treatment
-replace B12 and intrinsic factor
Pernicious Anemia untreated
-permanent CNS damage
Folate Anemia causes
-impaired DNA synthesis and destruction of hematopoietic cells
S/Sx Folate Anemia
- cachexia
- sever cheilosis/angular stomatitis (cracks corners of mouth)
Treatment of Folate Anemia
-replace folate
Iron-Deficiency Anemia
-most common
Iron-Deficiency Anemia S/Sx
- pale skin
- fatigue
- irritability
- dizzy
- wakness
- SOB
- brittle nails
- decreased appetite
- frontal headache
- spoon nails
- restless leg syndrome
- pica
Iron Gets absorbed in:
- duodenum
- broken down in stomach acid
Iron-Deficiency Anemia Treatment
- replace iron: food
- red meat, liver, raisins, spinach, broccoli, egg yolks
Sideroblastic Anemia
- associated with alcoholism and medication use
- hereditary factor
- RBC still have nucleus
Sideroblastic Anemia Profile
-Male>female
Sideroblastic Anemia: Pathophys
-iron not synthesized into hemoglobin
S/Sx Sideroblastic Anemia
- spleen/liver enlargement
- bronze skin
- arrhythmias
- presence of sideroblasts
Sideroblastics Anemia Treatment
- pyridoxin therapy (B6)
- Iron depletion therapy
- phlebotomies
hemolytic anemia
- body destroying RBC
- caused by immune, drugs etc
Treatment of hemolytic anemia
-steroids for immunohemolytic anemia
Post-Hemorrhacig Anemia
- acute blood loss
- loss >1000ml of blood
Post-Hemorrhacig Anemia causes
-GI bleed from NSAID use
Post-Hemorrhacig Anemia Treatment
-restoration of blood volume
Anemia of Chronic Disease: disease examples
- AIDS
- chronic inflammatory disorders (RA, SLE, hepatitis, inflam bowel conditions)
- chronic renal failure
Treatment of Anemia of Chronic Disease
-treat underlying disorder to address anemia
Medical Management of Anemia
-alleviate/control cause
PT Guidelines for Anemic pt
- ex’s under Dr. supervision
- anemia due to renal failure: max VO2 20% less than normal;
- Use RPE scale
- caution: chest pain
Sickle Cell Disease
- group of hereditary disorders characterized by an abnormal form of Hb within RBC
- RBC become sickle shaped after several cycles of oxygenation-deoxygenation
Sickle Cell Disease seen in:
- equitorial countries
- Africa
Sickle Cell Disease results in:
reduction of total # of RBC in blood or decrease in quality or quantity of Hb
S/Sx Sickle Cell Disease
- dactylitis (swelling fingers–clots in fingers/feet)
- spontaneous hemorrhages/infarctions
- pain (vascular occlusion)
- AVN of fem head
- osteomyelitis
- end stage renal failure
- blindness
- gallstones
- slenomegaly
- pneumonia
Extensive sickling can cause:
crisis
vasoocclusive crisis/thrombotic crisis
-cells obstruct blood flow
aplastic crisis
-profound anemia from deminished erythropoiesis
sequestration crisis
-large amts blood pooled in spleen and liver
hyperhemolytic crisis
- increased RBC lysis;
- can lead to death
Treatment of sickle cell disease
- address symptoms
- folate in diet
- bone marrow transplantation
Chest Syndrome
- sickled cells adhere to lung endothelium
- Sx: chest pain, SOB, fever, wheeing, cough, pulm HTN
- poor prognosis
Sickle cell disease under age 20
-stroke in 11%
Complications of Sickle cell disease
- hand and foot syndrome
- jaundice
- anemia
- autosplenectomy
- stroke
- chest syndrome
medical management of SCD
- transfusion
- bone marrow transplant
- stem cell transplant
- education
- emergency med attn
- pn control
- caution with ice: vasoconstriction
Prognosis of SCD
- life expectancy
- men: 42
- women: 48
SCD + Exercise
- low peak VO2
- low anaerobic threshold
- gas exchange abnormalities
Examples of Disorders of Abnormal Blood Component Levels
- Anemia
- Polycythemia
- Leukocytosis
- Leukopenia
- Thrombocytosis
- Thrombocytopenia
Polycythemia Vera
- Excess production of blood constituents (RBC, WBC, leukocytes) in the bone marrow
- causes increased blood volume and viscosity
Cause of Polycythemia Vera
- unknown
- likely acquired genetic alterations
- may have connection to intense radiation exposure
- common response to hypoxia (living at high altitudes)
Polycythemia causes increased risk for:
- thrombi
- angina
- extremely painful itching
Treatment of Polycythemia Vera
- reduce RBC and blood volume to prevent thrombosis
- phlebotomies
- radioactive phosphorus suppresses hematopoeisis/erythropoeisis
Side effects of radioactive phosphorus
- anemia
- leukopenia
- thrombocytopenia
Leukocytosis
- higher than normal leukocyte count
- normal protective response to physiologic stressors
- can occur with malignancies and hematological disorders
WBC Count in infectiouse diseases
->10,000 WBC/mm3
Leukopenia
- lower than normal leukocyte count
- increases risk for infection
Neutropenia
- lower than normal levels of neutrophils
- less than 2000/microliter
Neutropenia occurs with:
- prolonged infections
- autoimmune disorders
- chemotherapy
- defective neutrophil production or regulation
Thrombocytosis
- increased # of platelets
- over 750,000 is serious
- primary vs secondary
Thrombocytosis can increase risk of:
- thrombus
- but no symptoms
Primary Thrombocytosis
- “essential”
- due to myeloproliferative disease
Secondary Thrombocytosis
- “reactive”
- response to inflammatory process
Platelet Levels in Thrombocytosis
->750,000 platelets/mm3
Thrombocytopenia
- low platelet count
- <100,000 platelets/mm3
Thrombocytopenia can cause increased risk for:
-hemorrhage with trauma
______is the most common drug induced thrombocytopenia
-Heparin
Causes of Thrombocytopenia
- platelet destruction
- lack of platelet formation
-associated with congenital and acquired diseases
Leukemia
- abnormal proliferation of WBC in blood and other organs
- symptoms suggesting acute infection
- severe: enlarged lymph nodes or spleen
Leukemia Pathophys
- increased WBC production inhibits normal cell production
- causing: anemia, bleeding, infection
Types of Leukemia
- ALL
- AML
- Chronic Lymphocytic Leukemia
- Chronic Myelogenous Leukemia
Classification of Leukemia based on:
- acute vs chronic
- predominant cell type: lymphocytic, vs. myelogenous
Lymphocytic
-originates in bone marrow
-infiltrates other tissues
(lymphatic system)
Myelogeous
- myeloid stem cells (bone marrow)
- interfere with all blood cell maturation (hematopoeitic cells)
Acute Leukemia
- Blocking of cell-differentiation of cells (B cells)
- immature blast cells
- adults>children
ALL
- Acute Lymphoblastic Leukemia
- most prevalent leukemia in children
AML
-Acute Myelogenous Leukemia
Chronic Leukemia
- mature abnormal cells
- unknown cause
CML
- Chronic Myelogenous Leukemia
- common with radiation exposure
- genetic component
CLL
- Chronic Lymphocytic Leukemia
- most common leukemia in Western society
S/Sx Leukemia
- Infection
- fever
- bleeding
- anemia
- bone/joint pain
- neurologic manifestations
- enlarged spleen
- extreme fatigue
- weight loss
- lymphadenopathy
- night sweats
Leukemia treatment
- chemo
- remission induction therapy
- supportive care
- direct treatment of infection
- bone marrow transplant
- immunnotherapy-evolving
- Stem cell transplantation
- palliative care
Malignant Lymphomas
-Cancers in lymphatic system
- Hodgkin’s Disease
- Non-Hodgkin’s Lymphoma
HD
-Hodgkin’s Disease
HD characterized by presence of:
- Reed-Sternberg Cells (owl eyes)
- cell most likely the malignant site
HD Profile
- young adults
- peak 20s-30s
- 8% of all lymphomas
Risk Factors of HD
- HIV/AIDS
- Mononucleosis infection
- chronic illness
- immunosuppressants
- Cancer treatment
- SLE
- autoimmune diseases
- ulcerative colitis
- drug abuse
- obesity
HD S/Sx
- enlarged painless lymph nodes
- fatigue
- fever
- night sweats
- pruritis
- weight loss
- pulm/radicular symptoms (spread to CNS rare)
- progression–>immunodeficiency and infections
HD Treatment
- biopsy to stage
- radiation
- chemo
HD Prognosis
- good
- one of the most curable forms of cancer
NHL
- Non-hodgkin’s Lymphoma
- solid tumors from lymph system cells
NHL Risk Factors
- age
- gender (male)
- environment
- viral infection
- immunocompromise
S/Sx NHL
- variable
- starts with lymphadenopathy
NHL Diagnosis
-biopsy is gold standard
NHL Treatment
- chemo
- radiation
NHL Prognosis
-depends how wide spread
Hemophilia
-Type A-C
- A/B: X-linked recessive
- C: autosomal recessive
Hemophilia Type A
- hereditary
- cause deficiency in factor VIII
- most common
Hemophilia Type B
- Hereditary
- cause deficiency in factor IX
von Willebrand disease
- hereditary
- cause deficiency in vWF
- most common bleeding disorder
vWF
- von Willebrand Factor
- transports/facilitates action of factor VIII
Hemophilia Treatment
-transfusion of plasma enriched with factor VIII
Hemophilia S/Sx
- bleeding
- bruising
- arthropathy (due to bleeding in joints)
- intramuscular hemorrhage
Benefits of Exercise in Bleeding Disorders
- improve work capacity
- joint protection through shock absorption
- decrease frequency of bleeds
- temporarily increase levels of clotting factors (VIII)
- improve immune function
Bleedign Disorder S/Sx
- fatigue
- joint pain
