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
Q

What is in the DDX for Non-Hodgkin’s lymphoma?

A

Leukemia
Hodgkin’s Disease
Mononucleosis
Sarcoidosis

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26
Q

How is Non-Hodgkin’s lymphoma differentiated from Hodgkin’s lymphoma?

A

No, Reed-Steinberh cells in lymph nodes in Non- Hodgkin’s

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27
Q

Which type of lymphoma tends to be more localized?

A

Hodgkin’s Disease

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28
Q

What are the main subtypes of Non-Hodgkin’s Lymphoma?

A

B-Cell Lymphoma (90 %)
T- Cell Lymphoma (10%)
NK-Cell Lymphoma (rare, less than 10%)

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29
Q

What are the common signs and symptoms of Multiple Myeloma?

A

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

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30
Q

What might you see on labs in a patient with Multiple Myeloma?

A

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

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31
Q

What will you see on urinalysis in a patient with Multiple Myeloma?

A

Proteinuria- Bence Jones Proteins

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32
Q

What will you see on bone x-ray in a patient with Multiple Myeloma?

A

Punched out lesions

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33
Q

What is in the DDx for Multiple Myeloma?

A

Metastatic Carcinoma
Lymphoma
Sarcoidosis
Paget’s disease of the bone

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34
Q

What is babesiosis?

A

A tick-borne parasitic infection that infects red blood cells. Possible Lyme Disease co-infection

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35
Q

Where is babesiosis most commonly found?

A

Most common in Northeastern and upper Midwest part of the United States in the warmer months of the year

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36
Q

What are the common signs and symptoms of babesiosis?

A

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

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37
Q

How is babesiosis diagnosed?

A

Peripheral blood smear will show parasites serum PCR or serology

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38
Q

What countries are considered endemic for malaria?

A

Haiti/Dominican Republic
Mexico
Central and South America, except Chile and Uruguay
North Africa, except Egypt, Libya, Algeria and Morocco

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39
Q

How is malaria transmitted?

A

Female anopheles mosquito transmits Plasmodia to Humans

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40
Q

What are the common signs and symptoms of Malaria?

A

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

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41
Q

How is malaria diagnosed?

A

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

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42
Q

Can chloroquine, the drug of choice for Malaria treatment, be given to a pregnant woman?

A

Yes

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43
Q

What are some common causes of septicemia?

A

Pyelonephritis
Acute prostatitis
Pneumonia
Pancreatitis
Appendicitis
Diverticulitis
Central line IV Contamination

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44
Q

What should you do if you suspect septicemia in a patient?

A

Refer to ED

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45
Q

What labs would you expect to be ordered in the ED for suspected septicemia?

A

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

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46
Q

What is enlargement of lymph NODE due to infiltration of inflammatory cells during an infectious disease called?

A

Lymphadentitis

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47
Q

What are the common signs and symptoms of lymphadenitis?

A

Can be painful and tender
Fluctuant and warn node
Soft, firm and rubbery node

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48
Q

What are some of the diseases that might cause posterior cervical lymphadenitis?

A

TB
Lymphoma
Mononucleosis (EBV)
Viral Illness

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49
Q

What diseases might cause cervical lymphadenitis?

A

Lymphoma, Hodgkin’s; Mononucleosis (EBV), Strep pharyngitis

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50
Q

What disease might cause unilateral inguinal lymphadenitis?

A

Syphilis and LGV. Most of the other STIs cause bilateral LAO c

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51
Q

What diseases might cause epitrochlear lymphadenitis?

A

Hand infections
Sarcoidosis
Secondary Syphillis
Breast Cancer

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52
Q

What disease might cause axillary lymphadenitis?

A

Breast Cancer
Lymphoma
Systemic Lupus Erythematosus

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53
Q

What herb is indicated for lymphadenitis?

A

Phytolacca americana

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54
Q

What is an infection for the lymphatic system called?

A

Lymphangitis, usually due to cellulitis caused by Streptococcus pyogenes

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55
Q

What would you expect on a recent history for a case of lymphangitis?

A

Recent wound or cellulitis

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56
Q

What are the signs and symptoms of lymphangitis?

A

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

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57
Q

How is a diagnosis of lymphangitis made?

A

Clinically
CBC count and differential; leukocytosis
Blood Culture
Culture of any pus or open wounds that may form

58
Q

What is lymphedema?

A

Abnormal collection of protein-rich fluid in the interstitium resulting from obstruction of lymphatic drainage

59
Q

What are the causes of primary lymphedema?

A

Caused by congenital hypoplasia or aplasia of the peripheral lymphatics or by valvular incompetence

Genetic Disorder: Turner’s Syndrome

60
Q

What are the causes of secondary lymphedema?

A

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
Q

What are the clinical findings of lymphedema?

A

Classical non-pitting edema
Impaired limb mobility, dull sensation
Discomfort, possibly painful
Loss of normal contours

62
Q

What are some of the common signs and symptoms of anemia?

A

Weakness
Fatigue
Dizziness
Palpitations/tachycardia
Exertional Dyspnea
Tinnitus
Pallor
Headache

63
Q

In a CBC, what does the MCV indicate?

A

Average volume or size of RBC: useful to classify as macrocytic or microcytic

64
Q

In a CBC, what does an elevated MCV indicate?

A

Macrocytic Anemia

65
Q

In a CBC, what does a depressed MCV indicate?

A

Microcytic Anemia

66
Q

In a CBC, what does an elevated MCH indicate?

A

MCH: amount of hemoglobin in RBC

Macrocytic Anemia

67
Q

In a CBC, what does a depressed MCH indicate?

A

Microcytic Anemia

68
Q

What is the measure of size variation in RBCs or anisocytosis?

A

RDW : Red Cell Distribution Width

69
Q

What changes in the RBC indices would you see with iron deficiency anemia?

A

Decreased MCH, MCV, MCHC ( amount of hemoglobin as to size)

Increased RDW; poor red cell production

70
Q

What changes in the RBC indices you you see with B12/folate deficiency?

A

Increased MCV, MCH,
RDW increased
MCHC: Normal

71
Q

What changes in the RBC indices would you see with hemolytic/aplastic anemia?

A

Normal MCV (or slightly elevated), MCH, MCHC

Elevated RDW

72
Q

What are the microcytic/hypochromic anemias?

A

Iron Deficiency
Alpha and Beta Thalassemias
Chronic Lead poisoning
Sideroblastic anemia

73
Q

What are the normocytic/normochromic anemias?

A

Acute blood loss
Hemolytic anemia
Chronic Disease
Aplastic Anemia

74
Q

What are the macrocytic/hyperchromic anemias?

A

Vitamin B12 deficiency
Folate deficiency
Liver Disease
Pernicious anemia

75
Q

In which groups is Glucose-6- phosphate deficiency (G6PD) most common?

A

Males X-linked recessive and/or
Middle Eastern
Mediterraneans
African Americans
Asians

76
Q

What situations bring on an attack of hemolytic anemia in the G6PD patient?

A

Exposure to oxidizing foods: Fava beans, high doses of IV vitamin C
Bacterial or viral infections
Diabetic Ketoacidosis
Medication induced

77
Q

Which important antioxidant molecule is typically low in G6PD individuals due to pathway disruption?

A

Glutathione

78
Q

What are the signs and symptoms of glucose 6 phosphate dehydrogenase (G6PD) deficiency?

A

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
Q

What would you see on a peripheral blood smear with G6PD deficiency?

A

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
Q

Which drugs are contraindicated in someone with G6PD?

A

Anti-malarial drugs (Quinidine and Quinine)
Aspirin
Nitrofurantoin
NSAIDs
Ciprofloxacin
Sulfa Drugs (including Furosemide)

81
Q

What vitamin IV therapy is contraindicated someone with G6PD?

A

High Dose Vitamin C IV therapy

82
Q

What are the common signs and symptoms of hemochromatosis?

A

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
Q

At what age do you start to see symptoms develop from hemochromatosis?

A

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
Q

What is the classic tetrad of manifestation resulting from long term untreated hemochromatosis?

A

1) Liver cirrhosis leading to hepatocellular carcinoma
2) Diabetes mellitus
3) Hyperpigmentation of the skin
4) Cardiac Failure

85
Q

What would be in the DDx for hemochromatosis?

A

Chronic Hepatitis
Alcoholic Liver Disease
Thalassemia
RA or SLE
Lyme Disease
Fibromyalgia

86
Q

What do you order to diagnose someone with hemochromatosis?

A

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
Q

What are the standard treatments and dietary guidelines for someone with hemochromatosis?

A

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
Q

What populations are more at risk for developing sickle cell anemia?

A

African Americans
Eastern Mediterranean
Middle Eastern

89
Q

What are the common signs and symptoms for sickle cell anemia ?

A

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
Q

What is seen on peripheral blood smear in a patient with sickle cell disease?

A

Sickled-shaped cells, Howell- Jolly bodies, nucleated erythrocytes and cell fragments

91
Q

What type of thalassemia presents with severe anemia, usually leading to early mortality?

A

Beta Thalassemia Major

92
Q

What are the symptoms of thalassemia minor?

A

Asymptomatic or mildly affected

93
Q

People from what geographical background are more likely to be affected with thalassemia?

A

Mediterranean, Africans or SE Asian Descent

94
Q

How is thalassemia diagnosed?

A

Hypochromic, microcytic anemia
Peripheral smear: target cells, basophilic stippling (alpha) , nucleated red cells, marked aniso and poikilocytosis

95
Q

What do the RBC’s look like in a patient with ACUTE hemorrhagic anemia?

A

Normocytic, hypochromic

96
Q

What do the RBC’s look like in a patient with CHRONIC hemorrhagic anemia?

A

Microcytic, hypochromic

97
Q

Which anemia has an insidious onset with severe symptoms of anemia, skin pallor, ecchymosis, petechiae, fever, and headache?

A

Aplastic Anemia: Destruction of hematopoietic cells of the bone marrow leading to pancytopenia and hypocellular bone marrow

98
Q

What is one drug that can lead to a toxin/drug induced aplastic anemia?

A

Methotrexate

99
Q

What causes poikilocytosis?

A

Increase in abnormal RBCs in any shape, more than 10% of population

Any severe anemia

100
Q

What would you see on labs with aplastic anemia?

A

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
Q

What are some of the causes of hemolytic anemia?

A

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
Q

What will you see on a urine dipstick in hemolytic anemia?

A

Increased Urobilinogen

103
Q

What would you see on a peripehral smear of RBCs with hemolytic anemia?

A

Basophilic stippling
Schistocytosis
Heinz Bodies
Bite Cells

104
Q

What test is done for Rh factor and to assist in diagnosis for hemolytic anemias?

A

Indirect Coombs Test
-Detects antibodies in serum that can recognize antigens on RBCs

105
Q

What are Howell-Jolly bodies?

A

Nuclear remnants that appear after removal by the spleen. Seen in hyposplenism, hemolytic anemia, hereditary spherocytosis

106
Q

What are some of the lab abnormalities seen in hemolytic disease?

A

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
Q

What are the signs and symptoms of hemolytic anemia?

A

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
Q

What type of anemias have bite cells on peripheral blood smear?

A

Hemolytic Anemia
G6PD Deficiency Anemia
Oxidative Hemolysis: Drug Induced

109
Q

What does the peripheral blood smear look like with megaloblastic anemia?

A

Anisocytosis (blood cells of different size)
Enlarged, oval- shaped erythrocytes
and hypersegmented neutrophils

110
Q

What are the 3 subgroups of marcocytic anemias?

A

Megaloblastic
-B12 Deficiency
-Folate Deficiency
-Pernicious Anemia
Non-Megaloblastic
-Alcohol
-Hypothyroidism

111
Q

What are causes of macrocytic anemias?

A

B12/Folate Deficiency
Alcoholism
Liver Cirrohosis

112
Q

What might you see in a patients nails with iron deficiency anemia?

A

Spooning or Koilonychia (soft nails)

113
Q

You have an older patient with unexplained iron deficiency anemia. What type of diagnostic imaging should you recommend?

A

Upper endoscopy
Colonoscopy

114
Q

What kind of anemia does lead poisoning cause?

A

Microcytic and hypochromic anemia

115
Q

What do the RBC’s look like in a patient with anemia of chronic disease?

A

Normocytic, Normochromic

116
Q

What are some of the causes of anemia of chronic disease?

A

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
Q

RBC Indices in Various Conditions

A

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
Q

What is polycythemia vera?

A

Stem cell disorder (neoplastic bone marrow disorder)
A primary myeloproliferative neoplasm that results in elevated RBC, WBC, and platelet levels

119
Q

What causes secondary polycythemia vera?

A

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
Q

What are the signs and symptoms of polycythemia vera?

A

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
Q

Which of the polycythemias presents with low erythropoietin?

A

Polycythemia Vera

122
Q

What is Disseminated intravascular coagulation (DIC)?

A

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
Q

What are the most common causes of DIC?

A

Infectious (Bacterial, viral, fungal, parasitic)
Malignancy (Hematologic & Metastatic)
Obstetric (Placental Abruption, Eclampsia, Amniotic Fluid Embolism)
Trauma (Burns, accidents)
Transfusion (Hemolytic reactions)
Pancreatitis

124
Q

What kind of bacteria infections most commonly result in DIC?

A

Gram-negative: Klebsiella , Acinetobacter, Pseudomonas and E.coli

125
Q

What are the common signs and symptoms of DIC?

A

Profuse bleeding: Multiple ecchymosis and mucosal bleeding
Hypovolemia
Hypotension

126
Q

How is an early diagnosis of DIC made?

A

Thrombocytopenia
Prolonged PT and PTT
Low Plasma Fibrinogen
Increased D-Dimer

127
Q

What type of inheritance pattern do Hemophilia A and B exhibit?

A

X-linked recessive
Females are asymptomatic carriers

128
Q

Which factors are deficient in the 2 kinds of hemophilia?

A

A: Deficiency of functional plasma clotting factor VIII (most co

B: Factor IX deficiency

129
Q

How is a diagnosis of hemophilia made?

A

Prolonged PTT with a normal bleeding time and normal PT

Clotting Factor Assay

PT/INR: Extrinsic
PTT: Intrinsic

130
Q

What is a common symptoms of hemophilia?

A

Spontaneous or prolonged bleeding and in severe cases hemarthrosis

131
Q

What are some of the common signs and symptoms of Idiopathic Thrombocytopenia Purpura (ITP) in adults?

A

Sudden onset of petechiae, easy bruising, epistaxis

1) Purpura and petechiae
2) Mucosal Bleeding
3) Thrombocytopenia
4) Increased Platelet destruction

132
Q

What are some of the common causes of ITP in adults?

A

History of viral disease
HIV infection
SLE
CLL or Non-Hodgkin’s lymphoma
Drug induced
Toxin Exposure

133
Q

What are the common signs and symptoms of Vitamin K deficiency?

A

1) Bleeding more than appropriate in response to minor trauma
2) Easy Bruising
3) Mucosal Bleeding

134
Q

What population is Vitamin K deficiency seen in most commonly?

A

Pediatrics: Hemorrhagic disease of the newborn;

Vitamin K Dependent Factors: 2, 4, 9, 10

135
Q

What is von Willebrand’s Disease?

A

vWF disease is a defective platelet plug formation due to autosomal dominant defect in quantity or quality of von Willebrand Factor

136
Q

How is von Willebrand’s Diagnosed?

A

Prolonged PTT
Normal PT
Prolonged Bleeding time
Low total plasma vWF antigen

137
Q

What are the symptoms of von Willebrand’s Disease?

A

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
Q

What is the principle issue in porphyria?

A

Enzyme deficiencies in the heme production pathway; due to inborn errors of metabolism or exposure to environmental toxins or infectious agents

139
Q

What color is the urine in a patient with porphyria?

A

Red-Brown

140
Q

Are prophyria’s inherited or acquired?

A

Most commonly inherited- autosomal dominant except for erythropoietic protoporphyria which is autosomal recessive

141
Q

What system is most affected by acute intermittent porphyria?

A

Nervous system

142
Q

What should you know about erythropoietic protoporphyria and porphyria cutanea tarda?

A

Rare metabolic disorders that result in extreme photosensitivity