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Systems Path II Final > Hematopoietic and Lymphoid > Flashcards

Hematopoietic and Lymphoid Flashcards

1
Q

What is the formation and development of blood cells

A

Hematopoiesis

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2
Q

Hematopoiesis occurs in the marrow of long bones: tibia, femur in children or aduts

A

Children

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3
Q

Hematopoiesis occus in the pelvis, cranium, vertebral bodies, sternum, ribs in children or adults

A

Adults

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4
Q

What is extramedullary hematopoiesis

A

Formation of blood cells outside of the medullary cavity (spleen, liver, lymph nodes, thymus)

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5
Q

A RBC undergoing hemolysis will release what

A

Hemoglobin (Hb)

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6
Q

Erythropoiesis occurs where

A

In red bone marrow

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7
Q

What is a Reticulocyte

A

Immature RBC (1% of circulating RBCs)

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8
Q

What is Polycythemia

A

Inc. RBCs

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9
Q

What is anemia

A

Dec. RBCs

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10
Q

What is the reason for a dec. RBC production anemia

A

Marrow Failure

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11
Q

How can the kidneys affect blood cell production

A

Inc. erythropoietin (Inc. erythropoiesis up to 8x)

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12
Q

What are the common S/S of anemia

A

Pallor, fatigue, weakness/lassitude

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13
Q

Rate of onset and mechanism have a direct impact on what with anemia

A

Severity

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14
Q

What does hypoxia cause the release of

A

Erythropoietin (EPO)

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15
Q

What is hemodilution

A

Inc. in plasma

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16
Q

What is reticulocytosis

A

Inc. in reticulocytes

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17
Q

Accelerated RBC destruction, hemolysis and dec. RBC life span refer to what type of anemia

A

Hemolytic

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18
Q

Hemolytic Anemia: What type of defects are hereditary: abnormal RBC membranes, enzyme deficiency, disordered Hb synthesis

A

Intracorpuscular

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19
Q

Hemolytic Anemia: What type of defects are Acquired: antibodies, RBC trauma, infxns. (malaria)

A

Extracorpuscular

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20
Q

What type of hemolysis is inside circulation, physical or biochemical damage, and releases Hb

A

Intravascular hemolysis

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21
Q

What type of hemolysis occurs inside the spleen and liver, RBC damage, antibody opsonization, splenomegaly, jaundice AND is MC

A

Extravascular hemolysis

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22
Q

What is a Poikilocyte

A

Abnormally shaped RBCs (general term)

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23
Q

What is a Shistocyte

A

Irregularly shaped RBCs, from hemolysis

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24
Q

What is a Dacrocyte

A

Teardrop-shaped RBC

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25
Q

Which Hemolytic Anemia:
Abnorm. RBC membrane
Fragile spherocytes removed by spleen, mod. anemia
Poss aplastic crisis parovirus B19 (5ths disease)
TX: Splenectomy

A

Hereditary Spherocytosis

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26
Q

Why are sites of blood stasis particularly bad in an individual with Sickle Cell anemia

A

The dec. O2 leads to sickling

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27
Q

What type of mutation is Sickle cell

A

B-globin, autosomal recessive

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28
Q

Heterozygous Sickle cell will result in what

A

Sickle cell trait = carriers, but not affected 8% Af. americans

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29
Q

Homozygous Sickle cell will result in what

A

Sickle cell anemia/disease, 1/600 Af. Americans

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30
Q

Which Hemolytic Anemia:
Thrombosis, fever, malaise, chronic low-level pain
Splenomegaly/infarction, gallstones, priapism
Stunted growth, osseous distortion

A

Sickle Cell Anemia

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31
Q

Sickle cell related, What condition is from lung infections or PE produces pulmonary stasis leading to dec. O2 leading to thrombosis

A

Acute chest syndrome

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32
Q

Sickle cell related, What condition results from a cerebrovascular obstruction

A

Stroke

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33
Q

Sickle cell related, What condition can affect the heart, kidneys, and liver

A

Hypoxia-induced fatty changes

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34
Q

T/F: With Homozygous Sickle Cell 60% survive beyond the 5th decade

A

FALSE, 50% survive beyond the 5th decade

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35
Q

In sickle cell anemia, where is the MC place for and infarction

A

Bone marrow

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36
Q

What distinct sign can possibly be seen on xray in the vertebra

A

H-shaped vertebra aka Lincoln Log Vertebrae (10%)

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37
Q

Which Hemolytic Anemia:

Abnormal hemoglobin, mutated alpha or beta globin genes, autosomal recessive, seen most in areas of endemic malaria

A

Thalassemia

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38
Q

What does the mutation in thalassemia cause

A

excess of the opposite globin chain and damages RBCs (hemolysis) as well as erythroblasts

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39
Q

If you have to have beta thalassemia, which do you want to have

A

Minor, 1 allele, subtle/very mild if any symptoms

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40
Q

Severe hemolysis and anemia, extramedullary hematopoiesis, splenomegaly, stunted growth, bone marrow expansion/distortion describes what condition

A

Beta-thalassemia major, 2 alleles

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41
Q

T/F: Tx. may not be necessary for beta-thalassemia minor

A

True!

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42
Q

What is the Tx for beta-thalassemia major

A

Repeated transfusions and iron chelation OR Bone marrow transplant may cure

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43
Q

Even with Tx, once a person is in their 20s, what will most likely be their downfall

A

Lethal Dilated Cardiomyopathy

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44
Q

What 2 xray signs can be seen in beta-thalassemia major

A

“hair on end” and “lace-like trabeculation”

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45
Q

What distinct facial features may be seen in beta-thalassemia major

A

Chipmunk face

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46
Q

Which Hemolytic Anemia:

Abnormal Hb, Slight dec. O2 capacity, ineffective erythropoiesis, highly variable, determined by # of altered genes

A

Alpha-Thalassemia

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47
Q

G6PD is needed to make Glutathione (GSH), which is a powerful

A

Antioxidant, protects against oxidative stress

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48
Q

G6PD Deficiency is MC in

A

Males

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49
Q

What is significant about G6PD deficiency

A

Asymptomatic until exposed to oxidative stress (infxns. MC)

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50
Q

G6PD Bite cells vs Heinz bodies

A

Bite:splenic macrophages
Heinz: oxidized Hb, clump together

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51
Q

What is the Tx for G6PD deficiency

A

Depends on severity, but Id/terminate oxidative stress, blood transfusion, partial splenectomy

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52
Q

Which Hemolytic Anemia:
Dark urine upon waking
Acquired PIGA gene mutation
X chromosome, but can still affect females

A

Paroxysmal Nocturnal Hemoglobinuria

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53
Q

T/F: PNH is the MC mild/ chronic low-level anemia

A

True

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54
Q

People with PNH are at an inc. risk for what

A

venous thrombosis

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55
Q

What is the tx for PNH

A

antibodies that inhibit the MAC, or marrow transplant (curative)

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56
Q

How are Immunohemolytic anemias Dx.

A

Via direct Coombs antiglobulin test

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57
Q

Spontaneous and idiopathic describes what kind of stimuli in immunohemolytic anemias

A

Endogenous

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58
Q

Toxic exposure or ADRs describes what kind of stimuli in immunohemolytic anemias

A

Exogenous

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59
Q

What type of Immunohemolytic Anemia:

IgG opsonization, most are primary = idiopathic, 25% develop secondary

A

Warm antibody type

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60
Q

What type of Immunohemolytic Anemia:

Periphery, IgM opsonization prim or sec to infxn or B cells lymphoma

A

Cold antibody type

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61
Q

Which Hemolytic Anemia:
Repetitive physical trauma to RBCs, prosthetic heart valves (blender effect), narrowing of vessels, RBCs become shistocytes, MC asymptomatic

A

Traumatic Hemolysis

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62
Q

Plasmodium falciparum, protozoan, female anopheles mosquito, leads to what condition

A

Malaria

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63
Q

Where is malaria MC

A

Asia and Africa

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64
Q

Episodic shaking, chills, fever, joint pain, renal failure, splenomegaly, jaundice, symptom “showers” approx. every 48 hours all describe

65
Q

What type of malaria involves the CNS, seizures, convulsions, coma/death, lethal within days

A

Cerebral malaria

66
Q

Though drug resistance is building, what meds are used

A

Chloroquine, primaquine, chemotherapy

67
Q

What is the World’s MC nutritional deficiency as well as the MC cause of anemia

A

Iron deficiency anemia (10% developed, 25-50% developing)

68
Q

What % of the body’s iron is in Hb

69
Q

Where is Fe stored

A

Liver, spleen, marrow, skeletal m.

70
Q

What transports Fe

A

transferrin

71
Q

What is the MC cause of IDA in developING nations

A

Dec. dietary intake

72
Q

What is the MC cause of IDA in developED nations

A

Chronic blood loss

73
Q

What condition does this describe: Insidious onset, asypmto/mild, fatigue, pale skin, lack of energy, impaired cognition, dec. immunity, fingernail spooning, Pica

A

Iron Deficiency Anemia

74
Q

What anemia is characterized by a deficiency in folate and B12, leading to inadequate DNA replication

A

Megaloblastic Anemia, Folate and B12 are required for DNA synthesis

75
Q

Megaloblastic anemia can present with Pancytopenia, what is that

A

Anemia (dec. RBCs), Leukopenia (dec. WBCs), and Throbocytopenia (dec. platelets)

76
Q

Sore tongue and no neuralgic dysfunction with macrocytes found in peripheral blood would indicatae

A

Folate deficiency anemia (Rare)

77
Q

Other than DNA synthesis, what else is B12 important for

A

PNS and Spinal cord maintenance

78
Q

Although a dietary B12 deficiency is rare, who is at risk

A

Strict vegans

79
Q

What is the MC cause of B12 deficiency

A

Chronic malabsorption, autoimmune gastritis = dec intrinsic factor MC in elderly >70

80
Q

T/F: B12 injections will resolve the problems

A

FALSE, Neuologic recovery is unlikely

81
Q

Anemia of Chronic disease is MC among which group of people

A

hospitalized individuals

82
Q

What is known as “bone marrow failure” from suppression of myeloid stem cells with pancytopenia

A

Aplastic Anemia

83
Q

Half of aplastic anemias are idiopathic, which means what prognosis wise

A

Idiopathic = Worse prognosis

84
Q

What is attacking the marrow with autoimmune aplastic anemia

A

autoreactive T cells

85
Q

What do these progressive features describe:
Anemia: weakness, pallor, dyspnea
Thrombocytopenia (dec. platelets) leading to petechiae
Granulocytopenia: infxns
Splenomegaly “charactaristically absent”

A

Aplastic Anemia

86
Q

What is the Tx for aplastic anemia

A

Immunosuppressive meds ~80% respond

87
Q

Who has the best prognosis for aplastic anemia

A

<40 with no Hx. of transfusions

88
Q

Which anemia is described by: Mets to bone MC: breast lung, prostate
Granulomatous disease (TB, Bridges-Good syndrome)
Lipid storage disease (Niemann-Pick type C)
Anemia and thrombocytopenia
Dacrocytes (tear RBCs)
Tx. marrow transplant

A

Myelophthisic Anemia

89
Q

What is leukocytosis/penia

A

Inc./Dec. WBCs

90
Q

Neutrophils, Eosinophils, and Basophils are all what

A

Granulotyes

91
Q

This granulocyte is MC, arrives first, 5 lobes

A

Neutrophils

92
Q

This granulocyte deals with allergies, parasites, 2 lobes

A

Eosinophils

93
Q

This granulocyte is rare, and prevents excessive clots (heparin)

94
Q

Monocytes are the precursors to what

A

Macrophages

95
Q

What occupies most of the cell in a lymphocyte

96
Q

Lymphocytes and monocytes are what

A

Agranulocytes

97
Q

Reactive disorders of WBCs are MC caused by what

A

Microbial infxn

98
Q

Neoplastic WBC disorders make up what % of adult and childhood CA related deaths

A

9% adult, 40% childhood

99
Q

What is Neutropenia/Agranulocytosis (severe)

A

Dec. WBC production or Inc WBC destro

100
Q 
What condition:
Cause by EBV, HHV-4
Acute, self-limited 4-6 weeks
Splenomegaly, hepatitis
Infected B cells "atypical lymphocytes"
Dx. Monospot test for EBV bodies
Risk for various B cell malignancies (immunosuppression adds to risk)
A

Infectious Mononucleosis

101
Q

What is the difference between infectous mono in developING vs developED countries

A

Developing-kids, MC asympto

Developed-adolescent, MC symptomatic

102
Q

What % succumb to a mononucleosis infxn

103
Q

2 weeks after, lasting 2-4 months with 90% of its cases being pediatric, Irregular setllate necrotizing granulomas, what condition is caused by Bartonella henselae (self limiting)

A

Cat-Scratch Disease

104
Q

Non-Hodgkin lymphoma, Hodgkin lymphoma, and Multiple Myeloma are all what type of neoplasms

105
Q

Acute Myelogenous leukemia, Myelodysplastic syndromes, and Chronic Myeloproliferative Disorders are all what kind of neoplasms

106
Q

Langerhans Cells Histiocytosis is what kind of neoplasm

A

Histiocytic

107
Q

What neoplasm is described by:
Controversial classification, Lymphocytes, nodes, thymus, and spleen
Arise from a single transformed cell
Mets to spleen, liver, marrow

A

Lymphoid Neoplasm

108
Q

What is a WBC cancer involving marrow or blood

109
Q

What is a WBC cancer involving lymphatic tissues

110
Q

Acute leukemia’s onsets are described how

A

Sudden and Stormy onset

111
Q

How do acute leukemias MCly manifest

A

As Anemia (fatigue)

112
Q

Which condition: aggressive CA of lymphoblasts
Pre-B (marrow/peripheral blood, MC) or Pre-T (thymus) cells
80% of pediatric leukemias
MC Dx at 4, (15-20 for T)
Tx. Chemotherapy 80% cure

A

Acute Lymphoblastic Leukemia

113
Q

What is the difference btwn. leukemia and lymphoma

A 
Phoma = mass
kemia = spreading/in circulation
114
Q

Which condition is a CA of B cells combined w/ immunosuppression
MC leukemia of adulthood (MC Dx. at 60, males 2x)
Asymptomatic early, but gradually progresses

A

Chronic Lymphocytic Leukemia

115
Q

What is the difference btwn. CLL and SLL

A

CLL is MC and >4000 lymphocytes involving blood, Small lymphocytic lymphoma <4000, involves nodes

116
Q

In Chronic lymphocytic leukemia tumor cells displace marrow leading to what

A

Pancytopenia and suppression of B cells leading to immune dysregulation

117
Q

Prognosis of CLL

A

4-6 years, if becomes aggro approx 1 year survival

118
Q

What condition is a B cell cancer, with a nodular pattern, centrocytes present, lymphocytes with cleaved nuclei

A

Follicular Lymphoma

119
Q

Which condition accounts for 40% of adult non-Hodgkins lymphoma (NHL), >50 y/o, Incurable (marrow involved at Dx) variable prognosis 1-20 years, but avg. 7-9

A

Follicular lymphoma

120
Q

T/F: 40% of Follicular lymphomas progress into diffuse B cell lymphoma

121
Q

What condition: rare B cell CA, 4% of NHL
frequently involves other tissues at Dx. (marrow, spleen, liver), vague features, MC males >50, Incurable, aggro 3-5 years

A

Mantle Cell lymphoma

122
Q

What condition: B cell CA enlarged by 4x size, Rapidly-enlarging mass(es), may invade any organ, Tx. high dose chemo (rapidly fatal w/o, w/ 80% enter remission, 50% cured) MC lymphoma of adulthood, 50% of all NHL, MC 60 but can happen at any age, Hx. of EBV and immunosuppressants inc. risk

A

Diffuse Large B cell lymphoma

123
Q

What condition: B cell CA, classically affecting African children, high rates of B cell prolifer and apoptosis (starry sky histo pattern”, Fastest growing human tumor, MC extranodal (Af maxilla and mandible, US abdomen, GI, ovaries), Hx. of EBV and mutated MYC gene inc. risk

A

Burkitt Lymphoma

124
Q

T/F: Burkitt lymphoma has a good prognosis if Tx with aggressive chemotherapy, most children being cured

125
Q

What is the plasma cell CA, males of African descent about 70, “punched-out”, HA, dizzy, confusion, Renal failure (Bence-Jones proteins), malignant plasma cells from a single progenitor plasma cell, 4-6 years, incurable and progressive, Plasmapheresis/Stem cell transplant

A

Multiple Myeloma

126
Q

Where is the MC site of lytic lesions in multiple myeloma

A

Verterbral column (comp fx), ribs 44%, skull 41%, pelvis 28, femur 24 fx

127
Q

What is indicated by salt and pepper skull

A

Hyperparathyroidism

128
Q

What cells are large, with “owl-eye” nuclear appearance

A

Reed-Sternberg cells

129
Q

How is hodgkin lymphoma different from NHL

A

Reed-Sternberg cells, arises from a single node, spread to local nodes (predictable mets)

130
Q

Males ages 15-40 and >55 with a family Hx, previous EBV infxn (70%), exposure to agent orange, immunosuppresion are at the greatest risk for what

A

Hodgkin Lymphoma

131
Q

May be asymptomatic, but a painless single node, fever, night sweats, cachexia, anemia, splenomegaly, hepatomegaly, pruritis are all features of what

A

Hodgkin Lymphoma

132
Q

What kind of neoplasms MCly affect adults, from transformed myeloblasts, clonal proliferations replace marrow

A

Myeloid neoplasms

133
Q

Which myeloid neoplasm: Aggressive, MC Dx around 50, Common CA, immature blasts in marrow, displace marrow >20%, suppress hematopoiesis, may mimic ALL

A

Acute Myeloid Leukemia

134
Q

Pancytopenia, Tx. with chemotherapy, bone marrow/stem cell transplant, and transfusions, poor prognosis, 15-30% long term survival describes what condition

A

Acute Myeloid Leukemia

135
Q

Myelodysplastic syndromes are aka

A

Pre-leukemia

136
Q

Which myeloid neoplasm: is a CA of myeloblasts filling the marrow, Megaloblasts, 40% transform into AML, Cytopenia, monosomy or trixomy, 1-2 years, poor response to Tx

A

Myelodysplastic syndromes

137
Q

Which myeloid neoplasm: Group of indolent tumors
Hyperplastic myeloid progenitors, retain ability to differentiate
Spread to other organs

A

Chronic Myeloproliferative Disorders

138
Q

Which myeloid neoplasm/Chronic Myeloproliferative disorder: 20% of all leukemia cases
Adults MC 25-60
Leukocytosis MC inc. granulocytes and platelets
Insidious and slowly progressive
Extreme splenomegaly “dragging”
Red pulp of spleen extreme hematopoiesis “beefy app”
3 years, marrow xplant 70% curative, meds tyrosine kinase inhibitors

A

Chronic Myelogenous Leukemia (Philadelphia Ph chromo 95% of cases)

139
Q 
Which myeloid neoplasm/Chronic Myeloproliferative disorder: Inc. concentration of RBCs
Either relative (dec plasma), or Absolute (inc. total RBC mass)
A

Polycythemia

140
Q

What is Panmyelosis

A

Too many RBCs, WBCs, and platelets (severely elevated, focus on polycythemia, RBCs) seen in Polycythemia Vera

141
Q

Which myeloid neoplasm/Chronic Myeloproliferative disorder: Panmyelosis, JAK2 point mutation, Avg. Dx 60, polycythemia, dec EPO, Inc whole blood volume = inc viscosity

A

Polycythemia Vera (PCV)

142
Q

How is Polycythemia Vera Tx

A

Marrow transplant, chemo

Prognosis 10-20 post Dx, w/o Tx 3 years

143
Q

Which myeloid neoplasm/Chronic Myeloproliferative disorder: Diffuse fibrosis of bone marrow, Extramed. hemato, massive splenomegaly, subtle hepatomegaly, inefficient hemato, early spent phase, Dacrocytes, RARE 65 y/o, 4-5 years

A

Primary Myelofibrosis

144
Q

What are Histiocytes

A

Macrophages or dendritic cells

145
Q

What are Langerhans cells

A

dendritic cells of the skin/mucosa

146
Q

What are Birbeck Granules

A

“Tennis-racket” organelle

147
Q

Where is Unisystem Langerhans cell Histiocytosis MCly seen osseously

A

calvaria, ribs, femur

148
Q

Which conditions is seen mostly in young children, multifocal skin lesions and fever, hepatosplenomegaly, w/o Tx rapidly fatal, chemo 50% 5 year survival, may spontaneously remiss

A

Langerhans cell histiocytosis

149
Q

Which condition: systemic coagulation leading to widespread thrombi (lethal), widespread hemorrhage, Tx. anticoags or coags (directed by features)

A

Disseminated Intravascular Coagulation (DIC)

150
Q

What can the dec. platelets in Thrombocytopenia lead to

A

Bleeding tendency

151
Q

Which condition: Antibodies (IgG) attack platelets, possible CNS hemorrhage (rare), Tx splenectomy 65% remission

A

Immune Thrombocytopenic Purpura

152
Q

Which ITP is seen in children, self-limited, secondary to viral infxn

A

Acute Immune Thrombocytopenic Purpura

153
Q

Which ITP is insidious, ADRs, idiopathic, reproductive age females, easy bruising

A

Chronic Immune Thrombocytopenic Purpura

154
Q

Which condition: Autosomal dominant, Dysfunctional platelet adherence, MC inherited bleeding disorder 1%, bleeding gums, easy bruising, epstaxis, heavy menstruation, Tx. transfusions or meds to inc von Willebrand factor (avoid blood thinners)

A

von Willebrand Disease

155
Q

Which condition: Spontatneous bleeding and poor wound healing, X-linked recessive males MC, 30% sporadic, petechiae is absent, 1/5000 males, Dx dec coagulation factor VIII in serum, Tx. factor VIII infusion, clotting factor meds favorable prognosis

A

Hemophilia A

156
Q

Which condition: aka Christmas diease, X-linked, 1/20,000 males, Mutated factor IX severe def, Tx. infusion of coag factor IX

A

Hemophilia B

157
Q

Which condition: Reactive B cells in the thymus, Myasthenia Gravis, SLE, RA, Thymectomy is an early Tx option

A

Thymic Hyperplasia

158
Q

Which condition: Rare, MC affects adult or MG patients, Benign (encapsulated) or malignant, Asian, Hx. of EBV infxn, Carcinoma obstructive/invasive

Systems Path II Final (3 decks)

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