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Medical Conditions II > Hematologic Disorders > Flashcards

Hematologic Disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

examples of blood disorders

A
  • hemochromatosis
  • anemia
  • leukocytosis
  • leukopenia
  • leukemia
  • hodgkin’s
  • non-hodgkins lymphoma
  • polycythemia vera
  • thrombocytosis
  • thrombocytopenia
  • sickle cell disease
  • thalassemias
  • hemophilias
  • splenomegaly
  • multiple myeloma
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2
Q

Hemochromatosis

A
  • accum of excess iron in many organs
  • especially liver, pancreas, skin
  • leads to cell damage and functional insufficiency
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3
Q

Disorders of Iron Absorption

A

-Hemochromatosis

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4
Q

Early Symptoms of Hemocrhomatosis

A
  • fatigue
  • heart palpitations
  • imporence
  • loss menstrual periods
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5
Q

S/Sx Hemochromatosis

A
  • severe weakness/fatigue
  • joint pain/abdominal pain (2nd/3rd MCP)
  • signs liver disease, diabetes, heart probs
  • impotence, infertility, loss menstrual periods
  • porphyria, hepatitis
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6
Q

Hemochromatosis Blood Tests

A
  • elevated:
  • -transferrin saturation >50%
  • -serum ferritin >1000 ng/ml
  • -transaminase
  • -blood glucose
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7
Q

Later Symptoms of Hemochromatosis

A
  • grey/bronze skin
  • cirrhosis of liver
  • liver cancer
  • DM
  • hypopituitarism
  • decreased pit/gonadal function
  • joint disease
  • heart failure
  • abdominal pain
  • fatigue
  • certain infections
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8
Q

Disorderd of Erythrocytes

A

-Anemias

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9
Q

Anemia

A
  • reduction of total number of RBC in blood or decrease in quality/quantity of Hb
  • affects O2 transport
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10
Q

Anemias result from:

A
  • decreased RBC production
  • increased RBC destruction
  • combo
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11
Q

Anemia S/Sx

A
  • pallor
  • SOB
  • palpitations
  • lethargy
  • easily fatigued
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12
Q

decreased prod of RBC–>

A
  • altered Hb synthesis
  • altered DNA synthesis
  • bone marrow infiltration
  • red cell aplasia (fail to develop)
  • kidney disease (erythropoietin)
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13
Q

Anemia Pathophysiology:

A
  • decreased/defective production of RBC
  • increased RBC destruction
  • Blood loss
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14
Q

cause of RBC destruction

A

-infection causing hemolysis

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15
Q

Diagnostic Tests of Anemia

A
  • HCT/Hb
  • size of RBC
  • serum iron level-serum ferritin
  • iron-binding capacity in blood
  • blood factors needed to absorb vitamins
  • CBC
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16
Q

Types of Anemia

A
  • pernicious (lack B12)–macrocytic
  • folate–macrocytic
  • iron-deficiency
  • sideroblastic
  • hemolytic
  • post-hemorrhagic
  • anemia of chronic disease
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17
Q

Pernicious Anemia

A
  • autoimmune response
  • lack intrinsic factor for B12 absorption
  • possible genetic link
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18
Q

Pernicious Anemia more common in

A
  • northern europeans
  • female>males
  • genetic
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19
Q

Pernicious Anemia Sx

A
  • weakness
  • fatigue
  • paresthesias (feet/fingers)
  • difficulty walking
  • loss appetite
  • abdominal pain
  • atrophic glossitis
  • skin yellow
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20
Q

B12 needed for

A

-myelin in axons and SC

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21
Q

atrophic glossitis

A
  • beefy red tongue

- swollen with no center fold

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22
Q

Pernicious Anemia treatment

A

-replace B12 and intrinsic factor

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23
Q

Pernicious Anemia untreated

A

-permanent CNS damage

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24
Q

Folate Anemia causes

A

-impaired DNA synthesis and destruction of hematopoietic cells

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25
Q

S/Sx Folate Anemia

A
  • cachexia

- sever cheilosis/angular stomatitis (cracks corners of mouth)

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26
Q

Treatment of Folate Anemia

A

-replace folate

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27
Q

Iron-Deficiency Anemia

A

-most common

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28
Q

Iron-Deficiency Anemia S/Sx

A
  • pale skin
  • fatigue
  • irritability
  • dizzy
  • wakness
  • SOB
  • brittle nails
  • decreased appetite
  • frontal headache
  • spoon nails
  • restless leg syndrome
  • pica
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29
Q

Iron Gets absorbed in:

A
  • duodenum

- broken down in stomach acid

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30
Q

Iron-Deficiency Anemia Treatment

A
  • replace iron: food

- red meat, liver, raisins, spinach, broccoli, egg yolks

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31
Q

Sideroblastic Anemia

A
  • associated with alcoholism and medication use
  • hereditary factor
  • RBC still have nucleus
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32
Q

Sideroblastic Anemia Profile

A

-Male>female

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33
Q

Sideroblastic Anemia: Pathophys

A

-iron not synthesized into hemoglobin

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34
Q

S/Sx Sideroblastic Anemia

A
  • spleen/liver enlargement
  • bronze skin
  • arrhythmias
  • presence of sideroblasts
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35
Q

Sideroblastics Anemia Treatment

A
  • pyridoxin therapy (B6)
  • Iron depletion therapy
  • phlebotomies
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36
Q

hemolytic anemia

A
  • body destroying RBC

- caused by immune, drugs etc

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37
Q

Treatment of hemolytic anemia

A

-steroids for immunohemolytic anemia

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38
Q

Post-Hemorrhacig Anemia

A
  • acute blood loss

- loss >1000ml of blood

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39
Q

Post-Hemorrhacig Anemia causes

A

-GI bleed from NSAID use

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40
Q

Post-Hemorrhacig Anemia Treatment

A

-restoration of blood volume

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41
Q

Anemia of Chronic Disease: disease examples

A
  • AIDS
  • chronic inflammatory disorders (RA, SLE, hepatitis, inflam bowel conditions)
  • chronic renal failure
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42
Q

Treatment of Anemia of Chronic Disease

A

-treat underlying disorder to address anemia

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43
Q

Medical Management of Anemia

A

-alleviate/control cause

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44
Q

PT Guidelines for Anemic pt

A
  • ex’s under Dr. supervision
  • anemia due to renal failure: max VO2 20% less than normal;
  • Use RPE scale
  • caution: chest pain
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45
Q

Sickle Cell Disease

A
  • group of hereditary disorders characterized by an abnormal form of Hb within RBC
  • RBC become sickle shaped after several cycles of oxygenation-deoxygenation
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46
Q

Sickle Cell Disease seen in:

A
  • equitorial countries

- Africa

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47
Q

Sickle Cell Disease results in:

A

reduction of total # of RBC in blood or decrease in quality or quantity of Hb

48
Q

S/Sx Sickle Cell Disease

A
  • 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
49
Q

Extensive sickling can cause:

A

crisis

50
Q

vasoocclusive crisis/thrombotic crisis

A

-cells obstruct blood flow

51
Q

aplastic crisis

A

-profound anemia from deminished erythropoiesis

52
Q

sequestration crisis

A

-large amts blood pooled in spleen and liver

53
Q

hyperhemolytic crisis

A
  • increased RBC lysis;

- can lead to death

54
Q

Treatment of sickle cell disease

A
  • address symptoms
  • folate in diet
  • bone marrow transplantation
55
Q

Chest Syndrome

A
  • sickled cells adhere to lung endothelium
  • Sx: chest pain, SOB, fever, wheeing, cough, pulm HTN
  • poor prognosis
56
Q

Sickle cell disease under age 20

A

-stroke in 11%

57
Q

Complications of Sickle cell disease

A
  • hand and foot syndrome
  • jaundice
  • anemia
  • autosplenectomy
  • stroke
  • chest syndrome
58
Q

medical management of SCD

A
  • transfusion
  • bone marrow transplant
  • stem cell transplant
  • education
  • emergency med attn
  • pn control
  • caution with ice: vasoconstriction
59
Q

Prognosis of SCD

A
  • life expectancy
  • men: 42
  • women: 48
60
Q

SCD + Exercise

A
  • low peak VO2
  • low anaerobic threshold
  • gas exchange abnormalities
61
Q

Examples of Disorders of Abnormal Blood Component Levels

A
  • Anemia
  • Polycythemia
  • Leukocytosis
  • Leukopenia
  • Thrombocytosis
  • Thrombocytopenia
62
Q

Polycythemia Vera

A
  • Excess production of blood constituents (RBC, WBC, leukocytes) in the bone marrow
  • causes increased blood volume and viscosity
63
Q

Cause of Polycythemia Vera

A
  • unknown
  • likely acquired genetic alterations
  • may have connection to intense radiation exposure
  • common response to hypoxia (living at high altitudes)
64
Q

Polycythemia causes increased risk for:

A
  • thrombi
  • angina
  • extremely painful itching
65
Q

Treatment of Polycythemia Vera

A
  • reduce RBC and blood volume to prevent thrombosis
  • phlebotomies
  • radioactive phosphorus suppresses hematopoeisis/erythropoeisis
66
Q

Side effects of radioactive phosphorus

A
  • anemia
  • leukopenia
  • thrombocytopenia
67
Q

Leukocytosis

A
  • higher than normal leukocyte count
  • normal protective response to physiologic stressors
  • can occur with malignancies and hematological disorders
68
Q

WBC Count in infectiouse diseases

A

->10,000 WBC/mm3

69
Q

Leukopenia

A
  • lower than normal leukocyte count

- increases risk for infection

70
Q

Neutropenia

A
  • lower than normal levels of neutrophils

- less than 2000/microliter

71
Q

Neutropenia occurs with:

A
  • prolonged infections
  • autoimmune disorders
  • chemotherapy
  • defective neutrophil production or regulation
72
Q

Thrombocytosis

A
  • increased # of platelets
  • over 750,000 is serious
  • primary vs secondary
73
Q

Thrombocytosis can increase risk of:

A
  • thrombus

- but no symptoms

74
Q

Primary Thrombocytosis

A
  • “essential”

- due to myeloproliferative disease

75
Q

Secondary Thrombocytosis

A
  • “reactive”

- response to inflammatory process

76
Q

Platelet Levels in Thrombocytosis

A

->750,000 platelets/mm3

77
Q

Thrombocytopenia

A
  • low platelet count

- <100,000 platelets/mm3

78
Q

Thrombocytopenia can cause increased risk for:

A

-hemorrhage with trauma

79
Q

______is the most common drug induced thrombocytopenia

A

-Heparin

80
Q

Causes of Thrombocytopenia

A
  • platelet destruction
  • lack of platelet formation

-associated with congenital and acquired diseases

81
Q

Leukemia

A
  • abnormal proliferation of WBC in blood and other organs
  • symptoms suggesting acute infection
  • severe: enlarged lymph nodes or spleen
82
Q

Leukemia Pathophys

A
  • increased WBC production inhibits normal cell production

- causing: anemia, bleeding, infection

83
Q

Types of Leukemia

A
  • ALL
  • AML
  • Chronic Lymphocytic Leukemia
  • Chronic Myelogenous Leukemia
84
Q

Classification of Leukemia based on:

A
  • acute vs chronic

- predominant cell type: lymphocytic, vs. myelogenous

85
Q

Lymphocytic

A

-originates in bone marrow
-infiltrates other tissues
(lymphatic system)

86
Q

Myelogeous

A
  • myeloid stem cells (bone marrow)

- interfere with all blood cell maturation (hematopoeitic cells)

87
Q

Acute Leukemia

A
  • Blocking of cell-differentiation of cells (B cells)
  • immature blast cells
  • adults>children
88
Q

ALL

A
  • Acute Lymphoblastic Leukemia

- most prevalent leukemia in children

89
Q

AML

A

-Acute Myelogenous Leukemia

90
Q

Chronic Leukemia

A
  • mature abnormal cells

- unknown cause

91
Q

CML

A
  • Chronic Myelogenous Leukemia
  • common with radiation exposure
  • genetic component
92
Q

CLL

A
  • Chronic Lymphocytic Leukemia

- most common leukemia in Western society

93
Q

S/Sx Leukemia

A
  • Infection
  • fever
  • bleeding
  • anemia
  • bone/joint pain
  • neurologic manifestations
  • enlarged spleen
  • extreme fatigue
  • weight loss
  • lymphadenopathy
  • night sweats
94
Q

Leukemia treatment

A
  • chemo
  • remission induction therapy
  • supportive care
  • direct treatment of infection
  • bone marrow transplant
  • immunnotherapy-evolving
  • Stem cell transplantation
  • palliative care
95
Q

Malignant Lymphomas

A

-Cancers in lymphatic system

  • Hodgkin’s Disease
  • Non-Hodgkin’s Lymphoma
96
Q

HD

A

-Hodgkin’s Disease

97
Q

HD characterized by presence of:

A
  • Reed-Sternberg Cells (owl eyes)

- cell most likely the malignant site

98
Q

HD Profile

A
  • young adults
  • peak 20s-30s
  • 8% of all lymphomas
99
Q

Risk Factors of HD

A
  • HIV/AIDS
  • Mononucleosis infection
  • chronic illness
  • immunosuppressants
  • Cancer treatment
  • SLE
  • autoimmune diseases
  • ulcerative colitis
  • drug abuse
  • obesity
100
Q

HD S/Sx

A
  • enlarged painless lymph nodes
  • fatigue
  • fever
  • night sweats
  • pruritis
  • weight loss
  • pulm/radicular symptoms (spread to CNS rare)
  • progression–>immunodeficiency and infections
101
Q

HD Treatment

A
  • biopsy to stage
  • radiation
  • chemo
102
Q

HD Prognosis

A
  • good

- one of the most curable forms of cancer

103
Q

NHL

A
  • Non-hodgkin’s Lymphoma

- solid tumors from lymph system cells

104
Q

NHL Risk Factors

A
  • age
  • gender (male)
  • environment
  • viral infection
  • immunocompromise
105
Q

S/Sx NHL

A
  • variable

- starts with lymphadenopathy

106
Q

NHL Diagnosis

A

-biopsy is gold standard

107
Q

NHL Treatment

A
  • chemo

- radiation

108
Q

NHL Prognosis

A

-depends how wide spread

109
Q

Hemophilia

A

-Type A-C

  • A/B: X-linked recessive
  • C: autosomal recessive
110
Q

Hemophilia Type A

A
  • hereditary
  • cause deficiency in factor VIII
  • most common
111
Q

Hemophilia Type B

A
  • Hereditary

- cause deficiency in factor IX

112
Q

von Willebrand disease

A
  • hereditary
  • cause deficiency in vWF
  • most common bleeding disorder
113
Q

vWF

A
  • von Willebrand Factor

- transports/facilitates action of factor VIII

114
Q

Hemophilia Treatment

A

-transfusion of plasma enriched with factor VIII

115
Q

Hemophilia S/Sx

A
  • bleeding
  • bruising
  • arthropathy (due to bleeding in joints)
  • intramuscular hemorrhage
116
Q

Benefits of Exercise in Bleeding Disorders

A
  • improve work capacity
  • joint protection through shock absorption
  • decrease frequency of bleeds
  • temporarily increase levels of clotting factors (VIII)
  • improve immune function
117
Q

Bleedign Disorder S/Sx

A
  • fatigue

- joint pain

Medical Conditions II (34 decks)

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