Conditions of the Blood and Lymphatic System Flashcards

1
Q

What age group is usually affected with Acute lymphocytic leukemia (ALL)?

A

All the Little Leaguers : Children

ALL: lymphoid precursors proliferate and replace hematopoietic cells; arrest in early stage

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2
Q

What are the symptoms of ALL?

A

Dyspnea
Mucocutaneous bleeding, frequent nosebleeds
LAO
Systemic symptoms: Weakness, fatigue, pallor
Bone Pain

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3
Q

Who is usually affected with chronic lymphocytic leukemia (CLL)?

A

Cranky Late Lifers
>60 years old
M>F

CLL: accumulation of functional incompetent B cells

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4
Q

What is the onset of CLL like?

A

Insidious onset, it can take several years for symptoms to manifest

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5
Q

What are the symptoms of CLL?

A

LAO
Recurring infections
Mucocutaneous bleeding
Splenomegaly and hepatomegaly
Systemic symptoms: weakness, fatigue, mild pallor, night sweats

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6
Q

What will you see on CBC and peripheral blood smear in a patient with CLL?

A

Smudge Cells (fragile leukemic cells)
Lymphocytes are small and mature

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7
Q

What age group is most affected with chronic myelogenous leukemia (CML)?

A

Median age 65, onset 30-50 years old
CML: Increased proliferation of the granulocytic cell line without the loss of their capacity to differentiate

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8
Q

What is the prognosis of CML?

A

90% 5- year survival rate

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9
Q

What are the symptoms of CML?

A

Systemic symptoms: fatigue, weakness, anorexia, fever, night sweats
Abdominal fullness
Splenomegaly
Blast crisis: bone pain, fever, malaise, bleeding tendency

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10
Q

What is found in nearly 80% of cases of CML?

A

Philadelphia Chromosome (9:22; translocation)

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11
Q

What is in the DDX for leukemia?

A

Infection: TB, EBV, CMV
Lymphoma
Multiple Myeloma
Leukemoid Reaction

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12
Q

How are leukemias diagnostically differentiated?

A

Bone Marrow Aspiration

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13
Q

What is Hodgkin’s disease?

A

Malignant proliferation of lymphoid cells with Reed-Sternberg cells that are believed to arise from germinal center B-cells

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14
Q

What age group tends to be affected by Hodgkin’s disease?

A

Young adults 15-30; then again >50

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15
Q

How is Hodgkin’s disease affected by alcohol consumption?

A

There is intense pain in affected areas with any alcohol consumption

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16
Q

What are the signs and symptoms of Hodgkin’s Disease?

A

Systemic sxs: Weight loss, night sweats, low grade cyclical fever
Pruritus
Painless, palpable lymphadenopathy

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17
Q

In a lateral view CXR what does”filling of the retrosternal space” mean?

A

Heart is enlarged
Aortic aneurysm
Mediastinal Mass

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18
Q

How does Hodgkin’s lymphoma spread?

A

It spreads through lymph vessels from node to node. Late is the disease it can enter the blood stream and spread to organs

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19
Q

What is the one of the first signs after onset of Hodgkin’s Disease?

A

Cervical or mediastinal LAO with a single hard non-mobile, non-tender node

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20
Q

How is a diagnosis of Hodgkin’s disease made?

A

Lymph node biopsy showing the presence of Reed-Sternberg Cells

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21
Q

What is a sign of Hodgkin’s disease on chest x-ray?

A

Hilar or mediastinal widening

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22
Q

What are the symptoms of Non-Hodgkin’s lymphoma?

A

Rubbery, discrete, non-tender LAO in inguinal or cervical chains (other nodes can be affected)
2) Abdominal pain or swelling
3) Chest pain, coughing, dyspnea
4) Systemic symptoms: fatigue, weight loss, night sweats, fevers

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23
Q

Is Hodgkin’s or Non-Hodgkin’s more common?

A

Non- Hodgkins more common

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24
Q

What age group is most affected in Non-Hodgkin’s lymphoma?

A

Incidence increases with age

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25
What is in the DDX for Non-Hodgkin's lymphoma?
Leukemia Hodgkin's Disease Mononucleosis Sarcoidosis
26
How is Non-Hodgkin's lymphoma differentiated from Hodgkin's lymphoma?
No, Reed-Steinberh cells in lymph nodes in Non- Hodgkin's
27
Which type of lymphoma tends to be more localized?
Hodgkin's Disease
28
What are the main subtypes of Non-Hodgkin's Lymphoma?
B-Cell Lymphoma (90 %) T- Cell Lymphoma (10%) NK-Cell Lymphoma (rare, less than 10%)
29
What are the common signs and symptoms of Multiple Myeloma?
Multiple Myeloma: proliferation of monoclonal plasma cells, produce large amounts of IgG or IgA Recurrent Bacterial Infections Bone Pain- especially in spine and sternum Weakness or numbness in legs Osteolytic lesions Hypercalcemia Renal Failure Systemic Symptoms: Fatigue, weight loss, anorexia, nausea, anemia, brain fog
30
What might you see on labs in a patient with Multiple Myeloma?
CRAB Increased Calcium Renal Failure Anemia Bony Lesions Pancytopenia with a low reticulocyte count, elevated ESR , abnormal coagualtion, peripheral blood smear can show rouleaux formation- stacking of RBCs
31
What will you see on urinalysis in a patient with Multiple Myeloma?
Proteinuria- Bence Jones Proteins
32
What will you see on bone x-ray in a patient with Multiple Myeloma?
Punched out lesions
33
What is in the DDx for Multiple Myeloma?
Metastatic Carcinoma Lymphoma Sarcoidosis Paget's disease of the bone
34
What is babesiosis?
A tick-borne parasitic infection that infects red blood cells. Possible Lyme Disease co-infection
35
Where is babesiosis most commonly found?
Most common in Northeastern and upper Midwest part of the United States in the warmer months of the year
36
What are the common signs and symptoms of babesiosis?
Many cases are asymptomatic initially Gradual onset: fatigue, malaise, weakness Fever with chills and sweats Headaches No LAO Arthralgia Hepatospenomegaly Erythema migrans if concurrent infection with Lyme Disease
37
How is babesiosis diagnosed?
Peripheral blood smear will show parasites serum PCR or serology
38
What countries are considered endemic for malaria?
Haiti/Dominican Republic Mexico Central and South America, except Chile and Uruguay North Africa, except Egypt, Libya, Algeria and Morocco
39
How is malaria transmitted?
Female anopheles mosquito transmits Plasmodia to Humans
40
What are the common signs and symptoms of Malaria?
High fever (cyclic), shaking, chills ( due to systemic lysis of RBC) Hepatosplenomegaly and thrombocytopenia without leukocytosis Abdominal pain, diarrhea, myalgia, headache, and cough Jaundice Seizures
41
How is malaria diagnosed?
Peripheral blood smears examined at 12-24 hour intervals (3x) to rule out infection Thick smears diagnosis presence of parasites; thin smears diagnosis species-identification
42
Can chloroquine, the drug of choice for Malaria treatment, be given to a pregnant woman?
Yes
43
What are some common causes of septicemia?
Pyelonephritis Acute prostatitis Pneumonia Pancreatitis Appendicitis Diverticulitis Central line IV Contamination
44
What should you do if you suspect septicemia in a patient?
Refer to ED
45
What labs would you expect to be ordered in the ED for suspected septicemia?
CBC with differential, chemistry panels, serum lactate, coagulation studies, blood culture, UA with culture CBC, electrolytes, BUN, creatinine, liver enzymes, INR, PTT, blood cultures, urine culture and sensitivity, culture any wounds
46
What is enlargement of lymph NODE due to infiltration of inflammatory cells during an infectious disease called?
Lymphadentitis
47
What are the common signs and symptoms of lymphadenitis?
Can be painful and tender Fluctuant and warn node Soft, firm and rubbery node
48
What are some of the diseases that might cause posterior cervical lymphadenitis?
TB Lymphoma Mononucleosis (EBV) Viral Illness
49
What diseases might cause cervical lymphadenitis?
Lymphoma, Hodgkin's; Mononucleosis (EBV), Strep pharyngitis
50
What disease might cause unilateral inguinal lymphadenitis?
Syphilis and LGV. Most of the other STIs cause bilateral LAO c
51
What diseases might cause epitrochlear lymphadenitis?
Hand infections Sarcoidosis Secondary Syphillis Breast Cancer
52
What disease might cause axillary lymphadenitis?
Breast Cancer Lymphoma Systemic Lupus Erythematosus
53
What herb is indicated for lymphadenitis?
Phytolacca americana
54
What is an infection for the lymphatic system called?
Lymphangitis, usually due to cellulitis caused by Streptococcus pyogenes
55
What would you expect on a recent history for a case of lymphangitis?
Recent wound or cellulitis
56
What are the signs and symptoms of lymphangitis?
Deep reddening of the skin, warmth, lymphadenitis, and a raised boarder around the affected area May also have chills and a high fever along with moderate pain and swelling
57
How is a diagnosis of lymphangitis made?
Clinically CBC count and differential; leukocytosis Blood Culture Culture of any pus or open wounds that may form
58
What is lymphedema?
Abnormal collection of protein-rich fluid in the interstitium resulting from obstruction of lymphatic drainage
59
What are the causes of primary lymphedema?
Caused by congenital hypoplasia or aplasia of the peripheral lymphatics or by valvular incompetence Genetic Disorder: Turner's Syndrome
60
What are the causes of secondary lymphedema?
Lymphatic drainage is altered by an acquired blockage of the lymph norse or by disruption of the local lymphatic channel Infection: filariasis (disease caused by a mosquito-born nematode infection of the parasite Wucheria bancroft; results in permanent lymphedema of the limb) Maligant Infiltration Obesity Radiation/Surgery CHF, infection, trauma, burns, insect bites
61
What are the clinical findings of lymphedema?
Classical non-pitting edema Impaired limb mobility, dull sensation Discomfort, possibly painful Loss of normal contours
62
What are some of the common signs and symptoms of anemia?
Weakness Fatigue Dizziness Palpitations/tachycardia Exertional Dyspnea Tinnitus Pallor Headache
63
In a CBC, what does the MCV indicate?
Average volume or size of RBC: useful to classify as macrocytic or microcytic
64
In a CBC, what does an elevated MCV indicate?
Macrocytic Anemia
65
In a CBC, what does a depressed MCV indicate?
Microcytic Anemia
66
In a CBC, what does an elevated MCH indicate?
MCH: amount of hemoglobin in RBC Macrocytic Anemia
67
In a CBC, what does a depressed MCH indicate?
Microcytic Anemia
68
What is the measure of size variation in RBCs or anisocytosis?
RDW : Red Cell Distribution Width
69
What changes in the RBC indices would you see with iron deficiency anemia?
Decreased MCH, MCV, MCHC ( amount of hemoglobin as to size) Increased RDW; poor red cell production
70
What changes in the RBC indices you you see with B12/folate deficiency?
Increased MCV, MCH, RDW increased MCHC: Normal
71
What changes in the RBC indices would you see with hemolytic/aplastic anemia?
Normal MCV (or slightly elevated), MCH, MCHC Elevated RDW
72
What are the microcytic/hypochromic anemias?
Iron Deficiency Alpha and Beta Thalassemias Chronic Lead poisoning Sideroblastic anemia
73
What are the normocytic/normochromic anemias?
Acute blood loss Hemolytic anemia Chronic Disease Aplastic Anemia
74
What are the macrocytic/hyperchromic anemias?
Vitamin B12 deficiency Folate deficiency Liver Disease Pernicious anemia
75
In which groups is Glucose-6- phosphate deficiency (G6PD) most common?
Males X-linked recessive and/or Middle Eastern Mediterraneans African Americans Asians
76
What situations bring on an attack of hemolytic anemia in the G6PD patient?
Exposure to oxidizing foods: Fava beans, high doses of IV vitamin C Bacterial or viral infections Diabetic Ketoacidosis Medication induced
77
Which important antioxidant molecule is typically low in G6PD individuals due to pathway disruption?
Glutathione
78
What are the signs and symptoms of glucose 6 phosphate dehydrogenase (G6PD) deficiency?
Usually asymptomatic until oxidative challenge Fever Dark Urine Jaundice or icterus Tachycardia and SOB Splenomegaly Frequently presents with episodic hemolysis precipitated by: Sudden onset of back pain with hemoglobinuria, 2 to3 days after oxidative stress
79
What would you see on a peripheral blood smear with G6PD deficiency?
Heinz bodies Bites Cells Reticulocytosis Heinz bodies damage the RBC membranes causing intravascular hemolysis and are then phagocytosed by splenic macrophages creating "Bite cells"
80
Which drugs are contraindicated in someone with G6PD?
Anti-malarial drugs (Quinidine and Quinine) Aspirin Nitrofurantoin NSAIDs Ciprofloxacin Sulfa Drugs (including Furosemide)
81
What vitamin IV therapy is contraindicated someone with G6PD?
High Dose Vitamin C IV therapy
82
What are the common signs and symptoms of hemochromatosis?
Hemochromatosis: Excess iron storage Severe fatigue Erectile Dysfunction Arthritis Hepatomegaly Alopecia Koilonychia Cirrhosis Bronze Diabetes: Destruction of B-islet cells Dilated Cardiomyopathy Malabsorption Hypogonadism Arthralgia: Degenerative disease of the joints
83
At what age do you start to see symptoms develop from hemochromatosis?
Usually 30-50 years old. Males have have earlier presentation, females after menopause Diagnosis usually made in the 50s In women, diagnosis is made 10 to 20 years after menopause (menses causes loss of iron )
84
What is the classic tetrad of manifestation resulting from long term untreated hemochromatosis?
1) Liver cirrhosis leading to hepatocellular carcinoma 2) Diabetes mellitus 3) Hyperpigmentation of the skin 4) Cardiac Failure
85
What would be in the DDx for hemochromatosis?
Chronic Hepatitis Alcoholic Liver Disease Thalassemia RA or SLE Lyme Disease Fibromyalgia
86
What do you order to diagnose someone with hemochromatosis?
Serum ferritin, transferrin saturation, LFT, genetic testing for C282Y mutation in HFE gene Increased serum iron, reticulocyte Decreased total iron-binding capacity (TIBC) Decreased serum luteinizing hormone and follicle stimulating hormone
87
What are the standard treatments and dietary guidelines for someone with hemochromatosis?
Phlebotomy once or twice weekly until serum iron and ferritin normalizes; lifelong maintenance every 2 to 6 months -Increase intake of tannin containing foods to reduce absorption -Avoid Vitamin C containing foods with meals as it increases absorption -Avoid cooking in cast iron cookware -Avoid raw shellfish -Avoid alcohol
88
What populations are more at risk for developing sickle cell anemia?
African Americans Eastern Mediterranean Middle Eastern
89
What are the common signs and symptoms for sickle cell anemia ?
1) Manifest early life, usually before 6 months of age; jaundice within the first year 2) Painful vaso-occlusive crisis: especially in chest, skeleton and/or abdomen 3) Fatigue 4) Dyspnea on exertion 5) Growth and developmental delay 6) Dactylitis (hand-foot syndrome): Painful swelling of hands and feet due to bone infarctions (occurs in infants) 7) Anemia 8) Hepatomeagly 9) Splenomegaly
90
What is seen on peripheral blood smear in a patient with sickle cell disease?
Sickled-shaped cells, Howell- Jolly bodies, nucleated erythrocytes and cell fragments
91
What type of thalassemia presents with severe anemia, usually leading to early mortality?
Beta Thalassemia Major
92
What are the symptoms of thalassemia minor?
Asymptomatic or mildly affected
93
People from what geographical background are more likely to be affected with thalassemia?
Mediterranean, Africans or SE Asian Descent
94
How is thalassemia diagnosed?
Hypochromic, microcytic anemia Peripheral smear: target cells, basophilic stippling (alpha) , nucleated red cells, marked aniso and poikilocytosis
95
What do the RBC's look like in a patient with ACUTE hemorrhagic anemia?
Normocytic, hypochromic
96
What do the RBC's look like in a patient with CHRONIC hemorrhagic anemia?
Microcytic, hypochromic
97
Which anemia has an insidious onset with severe symptoms of anemia, skin pallor, ecchymosis, petechiae, fever, and headache?
Aplastic Anemia: Destruction of hematopoietic cells of the bone marrow leading to pancytopenia and hypocellular bone marrow
98
What is one drug that can lead to a toxin/drug induced aplastic anemia?
Methotrexate
99
What causes poikilocytosis?
Increase in abnormal RBCs in any shape, more than 10% of population Any severe anemia
100
What would you see on labs with aplastic anemia?
CBC -Pancytopenia ( reduction in the # of RBC and WBC, as well as platelets); thrombocytopenia -Decreased reticulocytes (<1% of total RBC count) *Reticulocytes are immature red blood cells that mature in the bone marrow
101
What are some of the causes of hemolytic anemia?
Premature destruction of RBCs Hereditary Acquired 1) Abnormalities in cell membrane structure 2) Result of bone marrow no longer compensating for loss of red cells due to hemolysis 3) Abnormal hemoglobin or enzymes 4) Autoimmune 5) Drugs/Toxins
102
What will you see on a urine dipstick in hemolytic anemia?
Increased Urobilinogen
103
What would you see on a peripehral smear of RBCs with hemolytic anemia?
Basophilic stippling Schistocytosis Heinz Bodies Bite Cells
104
What test is done for Rh factor and to assist in diagnosis for hemolytic anemias?
Indirect Coombs Test -Detects antibodies in serum that can recognize antigens on RBCs
105
What are Howell-Jolly bodies?
Nuclear remnants that appear after removal by the spleen. Seen in hyposplenism, hemolytic anemia, hereditary spherocytosis
106
What are some of the lab abnormalities seen in hemolytic disease?
1) Increased LDH due to hemolysis 2) Increase Bilirubin: usually indirect 3) Increase serum iron 4) Increase Urine Urobilinogen 5) Increase Plasma Hemoglobin 6) Increase Reticulocytes
107
What are the signs and symptoms of hemolytic anemia?
Usual anemia signs: fatigue, tachycardia, DOE Jaundice due to unconjugated hyperbilirubinemia Hepatosplenomegaly (Hyperplasia of splenic and liver macrophages) Dark urine (hemoglobinuria, bilirubin) Cholelithiasis (pigment stones) Iron overload with extravascular (hepatic and splenic macrophages) hemolysis Iron deficiency with intravascular (in blood vessels) hemolysis
108
What type of anemias have bite cells on peripheral blood smear?
Hemolytic Anemia G6PD Deficiency Anemia Oxidative Hemolysis: Drug Induced
109
What does the peripheral blood smear look like with megaloblastic anemia?
Anisocytosis (blood cells of different size) Enlarged, oval- shaped erythrocytes and hypersegmented neutrophils
110
What are the 3 subgroups of marcocytic anemias?
Megaloblastic -B12 Deficiency -Folate Deficiency -Pernicious Anemia Non-Megaloblastic -Alcohol -Hypothyroidism
111
What are causes of macrocytic anemias?
B12/Folate Deficiency Alcoholism Liver Cirrohosis
112
What might you see in a patients nails with iron deficiency anemia?
Spooning or Koilonychia (soft nails)
113
You have an older patient with unexplained iron deficiency anemia. What type of diagnostic imaging should you recommend?
Upper endoscopy Colonoscopy
114
What kind of anemia does lead poisoning cause?
Microcytic and hypochromic anemia
115
What do the RBC's look like in a patient with anemia of chronic disease?
Normocytic, Normochromic
116
What are some of the causes of anemia of chronic disease?
Autoimmune disorders: RA, SLE, Crohn's Disease and Ulcerative Colitis Cancer: Lymphoma Chronic Kidney Disease Long-term infections: HIV/AIDS, Hepatitis B/C, bacterial endocarditis
117
RBC Indices in Various Conditions
Iron Deficiency Anemia -Decreased MCV,MCH, MCHC -Increased RDW Chronic Inflammation -Decreased MCV, everything else normal Pernicious Anemia/ B12/Folate Deficiency -Increased MCV & RDW, Normal MCH, -High Normal MCHC Hemolytic Anemia -Normal/Increase MCV & MCH Normal MCHC, RDW
118
What is polycythemia vera?
Stem cell disorder (neoplastic bone marrow disorder) A primary myeloproliferative neoplasm that results in elevated RBC, WBC, and platelet levels
119
What causes secondary polycythemia vera?
Defined as an absolute increase in red blood cell mass that is caused by enhanced stimulation of red blood cell production Decreased ambient oxygen concentration: living in high altitude COPD/ Pulmonary Fibrosis, Sleep Apena, Exposure to CO, Impaired perfusion to the kidneys which stim. EPO: Hypertension, Blood doping, Congenital Heart (right to left shunt)
120
What are the signs and symptoms of polycythemia vera?
Plethora or a ruddy complexion of face palms due to vessel congestion Hepatosplenomegaly Pruritis after bathing Peptic Ulcer Disease Gout Epistaxis, GI Bleeding Weakness, headache, light-headedness, fatigue, dyspnea
121
Which of the polycythemias presents with low erythropoietin?
Polycythemia Vera
122
What is Disseminated intravascular coagulation (DIC)?
Systemic activation of blood coagulation, which results in generation and deposition of fibrin, leading to uncontrolled microvascular coagulation and depletion of platelets, coagulation factors and fibrinogen lead to a risk of life-threatening hemorrhage
123
What are the most common causes of DIC?
Infectious (Bacterial, viral, fungal, parasitic) Malignancy (Hematologic & Metastatic) Obstetric (Placental Abruption, Eclampsia, Amniotic Fluid Embolism) Trauma (Burns, accidents) Transfusion (Hemolytic reactions) Pancreatitis
124
What kind of bacteria infections most commonly result in DIC?
Gram-negative: Klebsiella , Acinetobacter, Pseudomonas and E.coli
125
What are the common signs and symptoms of DIC?
Profuse bleeding: Multiple ecchymosis and mucosal bleeding Hypovolemia Hypotension
126
How is an early diagnosis of DIC made?
Thrombocytopenia Prolonged PT and PTT Low Plasma Fibrinogen Increased D-Dimer
127
What type of inheritance pattern do Hemophilia A and B exhibit?
X-linked recessive Females are asymptomatic carriers
128
Which factors are deficient in the 2 kinds of hemophilia?
A: Deficiency of functional plasma clotting factor VIII (most co B: Factor IX deficiency
129
How is a diagnosis of hemophilia made?
Prolonged PTT with a normal bleeding time and normal PT Clotting Factor Assay PT/INR: Extrinsic PTT: Intrinsic
130
What is a common symptoms of hemophilia?
Spontaneous or prolonged bleeding and in severe cases hemarthrosis
131
What are some of the common signs and symptoms of Idiopathic Thrombocytopenia Purpura (ITP) in adults?
Sudden onset of petechiae, easy bruising, epistaxis 1) Purpura and petechiae 2) Mucosal Bleeding 3) Thrombocytopenia 4) Increased Platelet destruction
132
What are some of the common causes of ITP in adults?
History of viral disease HIV infection SLE CLL or Non-Hodgkin's lymphoma Drug induced Toxin Exposure
133
What are the common signs and symptoms of Vitamin K deficiency?
1) Bleeding more than appropriate in response to minor trauma 2) Easy Bruising 3) Mucosal Bleeding
134
What population is Vitamin K deficiency seen in most commonly?
Pediatrics: Hemorrhagic disease of the newborn; Vitamin K Dependent Factors: 2, 4, 9, 10
135
What is von Willebrand's Disease?
vWF disease is a defective platelet plug formation due to autosomal dominant defect in quantity or quality of von Willebrand Factor
136
How is von Willebrand's Diagnosed?
Prolonged PTT Normal PT Prolonged Bleeding time Low total plasma vWF antigen
137
What are the symptoms of von Willebrand's Disease?
Easy bruising Excessive bleeding from wounds, dental surgery Menorrhagia Increased bleeding time and increase PTT (necause vWF stabilizes factor VIII) Decreased Factor VIII Platelet count normal Decreased von Willebrand antigen
138
What is the principle issue in porphyria?
Enzyme deficiencies in the heme production pathway; due to inborn errors of metabolism or exposure to environmental toxins or infectious agents
139
What color is the urine in a patient with porphyria?
Red-Brown
140
Are prophyria's inherited or acquired?
Most commonly inherited- autosomal dominant except for erythropoietic protoporphyria which is autosomal recessive
141
What system is most affected by acute intermittent porphyria?
Nervous system
142
What should you know about erythropoietic protoporphyria and porphyria cutanea tarda?
Rare metabolic disorders that result in extreme photosensitivity