Hematopoietic and Lymphoid Flashcards
(158 cards)
1
Q
What is the formation and development of blood cells
A
Hematopoiesis
2
Q
Hematopoiesis occurs in the marrow of long bones: tibia, femur in children or aduts
A
Children
3
Q
Hematopoiesis occus in the pelvis, cranium, vertebral bodies, sternum, ribs in children or adults
A
Adults
4
Q
What is extramedullary hematopoiesis
A
Formation of blood cells outside of the medullary cavity (spleen, liver, lymph nodes, thymus)
5
Q
A RBC undergoing hemolysis will release what
A
Hemoglobin (Hb)
6
Q
Erythropoiesis occurs where
A
In red bone marrow
7
Q
What is a Reticulocyte
A
Immature RBC (1% of circulating RBCs)
8
Q
What is Polycythemia
A
Inc. RBCs
9
Q
What is anemia
A
Dec. RBCs
10
Q
What is the reason for a dec. RBC production anemia
A
Marrow Failure
11
Q
How can the kidneys affect blood cell production
A
Inc. erythropoietin (Inc. erythropoiesis up to 8x)
12
Q
What are the common S/S of anemia
A
Pallor, fatigue, weakness/lassitude
13
Q
Rate of onset and mechanism have a direct impact on what with anemia
A
Severity
14
Q
What does hypoxia cause the release of
A
Erythropoietin (EPO)
15
Q
What is hemodilution
A
Inc. in plasma
16
Q
What is reticulocytosis
A
Inc. in reticulocytes
17
Q
Accelerated RBC destruction, hemolysis and dec. RBC life span refer to what type of anemia
A
Hemolytic
18
Q
Hemolytic Anemia: What type of defects are hereditary: abnormal RBC membranes, enzyme deficiency, disordered Hb synthesis
A
Intracorpuscular
19
Q
Hemolytic Anemia: What type of defects are Acquired: antibodies, RBC trauma, infxns. (malaria)
A
Extracorpuscular
20
Q
What type of hemolysis is inside circulation, physical or biochemical damage, and releases Hb
A
Intravascular hemolysis
21
Q
What type of hemolysis occurs inside the spleen and liver, RBC damage, antibody opsonization, splenomegaly, jaundice AND is MC
A
Extravascular hemolysis
22
Q
What is a Poikilocyte
A
Abnormally shaped RBCs (general term)
23
Q
What is a Shistocyte
A
Irregularly shaped RBCs, from hemolysis
24
Q
What is a Dacrocyte
A
Teardrop-shaped RBC
25
Which Hemolytic Anemia:
Abnorm. RBC membrane
Fragile spherocytes removed by spleen, mod. anemia
Poss aplastic crisis parovirus B19 (5ths disease)
TX: Splenectomy
Hereditary Spherocytosis
26
Why are sites of blood stasis particularly bad in an individual with Sickle Cell anemia
The dec. O2 leads to sickling
27
What type of mutation is Sickle cell
B-globin, autosomal recessive
28
Heterozygous Sickle cell will result in what
Sickle cell trait = carriers, but not affected 8% Af. americans
29
Homozygous Sickle cell will result in what
Sickle cell anemia/disease, 1/600 Af. Americans
30
Which Hemolytic Anemia:
Thrombosis, fever, malaise, chronic low-level pain
Splenomegaly/infarction, gallstones, priapism
Stunted growth, osseous distortion
Sickle Cell Anemia
31
Sickle cell related, What condition is from lung infections or PE produces pulmonary stasis leading to dec. O2 leading to thrombosis
Acute chest syndrome
32
Sickle cell related, What condition results from a cerebrovascular obstruction
Stroke
33
Sickle cell related, What condition can affect the heart, kidneys, and liver
Hypoxia-induced fatty changes
34
T/F: With Homozygous Sickle Cell 60% survive beyond the 5th decade
FALSE, 50% survive beyond the 5th decade
35
In sickle cell anemia, where is the MC place for and infarction
Bone marrow
36
What distinct sign can possibly be seen on xray in the vertebra
H-shaped vertebra aka Lincoln Log Vertebrae (10%)
37
Which Hemolytic Anemia:
| Abnormal hemoglobin, mutated alpha or beta globin genes, autosomal recessive, seen most in areas of endemic malaria
Thalassemia
38
What does the mutation in thalassemia cause
excess of the opposite globin chain and damages RBCs (hemolysis) as well as erythroblasts
39
If you have to have beta thalassemia, which do you want to have
Minor, 1 allele, subtle/very mild if any symptoms
40
Severe hemolysis and anemia, extramedullary hematopoiesis, splenomegaly, stunted growth, bone marrow expansion/distortion describes what condition
Beta-thalassemia major, 2 alleles
41
T/F: Tx. may not be necessary for beta-thalassemia minor
True!
42
What is the Tx for beta-thalassemia major
Repeated transfusions and iron chelation OR Bone marrow transplant may cure
43
Even with Tx, once a person is in their 20s, what will most likely be their downfall
Lethal Dilated Cardiomyopathy
44
What 2 xray signs can be seen in beta-thalassemia major
"hair on end" and "lace-like trabeculation"
45
What distinct facial features may be seen in beta-thalassemia major
Chipmunk face
46
Which Hemolytic Anemia:
| Abnormal Hb, Slight dec. O2 capacity, ineffective erythropoiesis, highly variable, determined by # of altered genes
Alpha-Thalassemia
47
G6PD is needed to make Glutathione (GSH), which is a powerful
Antioxidant, protects against oxidative stress
48
G6PD Deficiency is MC in
Males
49
What is significant about G6PD deficiency
Asymptomatic until exposed to oxidative stress (infxns. MC)
50
G6PD Bite cells vs Heinz bodies
Bite:splenic macrophages
Heinz: oxidized Hb, clump together
51
What is the Tx for G6PD deficiency
Depends on severity, but Id/terminate oxidative stress, blood transfusion, partial splenectomy
52
Which Hemolytic Anemia:
Dark urine upon waking
Acquired PIGA gene mutation
X chromosome, but can still affect females
Paroxysmal Nocturnal Hemoglobinuria
53
T/F: PNH is the MC mild/ chronic low-level anemia
True
54
People with PNH are at an inc. risk for what
venous thrombosis
55
What is the tx for PNH
antibodies that inhibit the MAC, or marrow transplant (curative)
56
How are Immunohemolytic anemias Dx.
Via direct Coombs antiglobulin test
57
Spontaneous and idiopathic describes what kind of stimuli in immunohemolytic anemias
Endogenous
58
Toxic exposure or ADRs describes what kind of stimuli in immunohemolytic anemias
Exogenous
59
What type of Immunohemolytic Anemia:
| IgG opsonization, most are primary = idiopathic, 25% develop secondary
Warm antibody type
60
What type of Immunohemolytic Anemia:
| Periphery, IgM opsonization prim or sec to infxn or B cells lymphoma
Cold antibody type
61
Which Hemolytic Anemia:
Repetitive physical trauma to RBCs, prosthetic heart valves (blender effect), narrowing of vessels, RBCs become shistocytes, MC asymptomatic
Traumatic Hemolysis
62
Plasmodium falciparum, protozoan, female anopheles mosquito, leads to what condition
Malaria
63
Where is malaria MC
Asia and Africa
64
Episodic shaking, chills, fever, joint pain, renal failure, splenomegaly, jaundice, symptom "showers" approx. every 48 hours all describe
Malaria
65
What type of malaria involves the CNS, seizures, convulsions, coma/death, lethal within days
Cerebral malaria
66
Though drug resistance is building, what meds are used
Chloroquine, primaquine, chemotherapy
67
What is the World's MC nutritional deficiency as well as the MC cause of anemia
Iron deficiency anemia (10% developed, 25-50% developing)
68
What % of the body's iron is in Hb
80%
69
Where is Fe stored
Liver, spleen, marrow, skeletal m.
70
What transports Fe
transferrin
71
What is the MC cause of IDA in developING nations
Dec. dietary intake
72
What is the MC cause of IDA in developED nations
Chronic blood loss
73
What condition does this describe: Insidious onset, asypmto/mild, fatigue, pale skin, lack of energy, impaired cognition, dec. immunity, *fingernail spooning*, Pica
Iron Deficiency Anemia
74
What anemia is characterized by a deficiency in folate and B12, leading to inadequate DNA replication
Megaloblastic Anemia, Folate and B12 are required for DNA synthesis
75
Megaloblastic anemia can present with Pancytopenia, what is that
Anemia (dec. RBCs), Leukopenia (dec. WBCs), and Throbocytopenia (dec. platelets)
76
Sore tongue and no neuralgic dysfunction with macrocytes found in peripheral blood would indicatae
Folate deficiency anemia (Rare)
77
Other than DNA synthesis, what else is B12 important for
PNS and Spinal cord maintenance
78
Although a dietary B12 deficiency is rare, who is at risk
Strict vegans
79
What is the MC cause of B12 deficiency
Chronic malabsorption, autoimmune gastritis = dec intrinsic factor MC in elderly >70
80
T/F: B12 injections will resolve the problems
FALSE, Neuologic recovery is unlikely
81
Anemia of Chronic disease is MC among which group of people
hospitalized individuals
82
What is known as "bone marrow failure" from suppression of myeloid stem cells with pancytopenia
Aplastic Anemia
83
Half of aplastic anemias are idiopathic, which means what prognosis wise
Idiopathic = Worse prognosis
84
What is attacking the marrow with autoimmune aplastic anemia
autoreactive T cells
85
What do these progressive features describe:
Anemia: weakness, pallor, dyspnea
Thrombocytopenia (dec. platelets) leading to petechiae
Granulocytopenia: infxns
Splenomegaly "charactaristically absent"
Aplastic Anemia
86
What is the Tx for aplastic anemia
Immunosuppressive meds ~80% respond
87
Who has the best prognosis for aplastic anemia
<40 with no Hx. of transfusions
88
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
Myelophthisic Anemia
89
What is leukocytosis/penia
Inc./Dec. WBCs
90
Neutrophils, Eosinophils, and Basophils are all what
Granulotyes
91
This granulocyte is MC, arrives first, 5 lobes
Neutrophils
92
This granulocyte deals with allergies, parasites, 2 lobes
Eosinophils
93
This granulocyte is rare, and prevents excessive clots (heparin)
Basophils
94
Monocytes are the precursors to what
Macrophages
95
What occupies most of the cell in a lymphocyte
Nuclei
96
Lymphocytes and monocytes are what
Agranulocytes
97
Reactive disorders of WBCs are MC caused by what
Microbial infxn
98
Neoplastic WBC disorders make up what % of adult and childhood CA related deaths
9% adult, 40% childhood
99
What is Neutropenia/Agranulocytosis (severe)
Dec. WBC production or Inc WBC destro
100
```
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)
```
Infectious Mononucleosis
101
What is the difference between infectous mono in developING vs developED countries
Developing-kids, MC asympto
| Developed-adolescent, MC symptomatic
102
What % succumb to a mononucleosis infxn
50%
103
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)
Cat-Scratch Disease
104
Non-Hodgkin lymphoma, Hodgkin lymphoma, and Multiple Myeloma are all what type of neoplasms
Lymphoid
105
Acute Myelogenous leukemia, Myelodysplastic syndromes, and Chronic Myeloproliferative Disorders are all what kind of neoplasms
Myeloid
106
Langerhans Cells Histiocytosis is what kind of neoplasm
Histiocytic
107
What neoplasm is described by:
Controversial classification, Lymphocytes, nodes, thymus, and spleen
Arise from a single transformed cell
Mets to spleen, liver, marrow
Lymphoid Neoplasm
108
What is a WBC cancer involving marrow or blood
Leukemia
109
What is a WBC cancer involving lymphatic tissues
Lymphoma
110
Acute leukemia's onsets are described how
Sudden and Stormy onset
111
How do acute leukemias MCly manifest
As Anemia (fatigue)
112
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
Acute Lymphoblastic Leukemia
113
What is the difference btwn. leukemia and lymphoma
```
Phoma = mass
kemia = spreading/in circulation
```
114
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
Chronic Lymphocytic Leukemia
115
What is the difference btwn. CLL and SLL
CLL is MC and >4000 lymphocytes involving blood, Small lymphocytic lymphoma <4000, involves nodes
116
In Chronic lymphocytic leukemia tumor cells displace marrow leading to what
Pancytopenia and suppression of B cells leading to immune dysregulation
117
Prognosis of CLL
4-6 years, if becomes aggro approx 1 year survival
118
What condition is a B cell cancer, with a nodular pattern, centrocytes present, lymphocytes with cleaved nuclei
Follicular Lymphoma
119
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
Follicular lymphoma
120
T/F: 40% of Follicular lymphomas progress into diffuse B cell lymphoma
True
121
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
Mantle Cell lymphoma
122
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
Diffuse Large B cell lymphoma
123
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
Burkitt Lymphoma
124
T/F: Burkitt lymphoma has a good prognosis if Tx with aggressive chemotherapy, most children being cured
True
125
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
Multiple Myeloma
126
Where is the MC site of lytic lesions in multiple myeloma
Verterbral column (comp fx), ribs 44%, skull 41%, pelvis 28, femur 24 fx
127
What is indicated by salt and pepper skull
Hyperparathyroidism
128
What cells are large, with "owl-eye" nuclear appearance
Reed-Sternberg cells
129
How is hodgkin lymphoma different from NHL
Reed-Sternberg cells, arises from a single node, spread to local nodes (predictable mets)
130
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
Hodgkin Lymphoma
131
May be asymptomatic, but a painless single node, fever, night sweats, cachexia, anemia, splenomegaly, hepatomegaly, pruritis are all features of what
Hodgkin Lymphoma
132
What kind of neoplasms MCly affect adults, from transformed myeloblasts, clonal proliferations replace marrow
Myeloid neoplasms
133
Which myeloid neoplasm: Aggressive, MC Dx around 50, Common CA, immature blasts in marrow, displace marrow >20%, suppress hematopoiesis, may mimic ALL
Acute Myeloid Leukemia
134
Pancytopenia, Tx. with chemotherapy, bone marrow/stem cell transplant, and transfusions, poor prognosis, 15-30% long term survival describes what condition
Acute Myeloid Leukemia
135
Myelodysplastic syndromes are aka
Pre-leukemia
136
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
Myelodysplastic syndromes
137
Which myeloid neoplasm: Group of indolent tumors
Hyperplastic myeloid progenitors, retain ability to differentiate
Spread to other organs
Chronic Myeloproliferative Disorders
138
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
Chronic Myelogenous Leukemia (Philadelphia Ph chromo 95% of cases)
139
```
Which myeloid neoplasm/Chronic Myeloproliferative disorder: Inc. concentration of RBCs
Either relative (dec plasma), or Absolute (inc. total RBC mass)
```
Polycythemia
140
What is Panmyelosis
Too many RBCs, WBCs, and platelets (severely elevated, focus on polycythemia, RBCs) seen in Polycythemia Vera
141
Which myeloid neoplasm/Chronic Myeloproliferative disorder: Panmyelosis, JAK2 point mutation, Avg. Dx 60, polycythemia, dec EPO, Inc whole blood volume = inc viscosity
Polycythemia Vera (PCV)
142
How is Polycythemia Vera Tx
Marrow transplant, chemo
| Prognosis 10-20 post Dx, w/o Tx 3 years
143
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
Primary Myelofibrosis
144
What are Histiocytes
Macrophages or dendritic cells
145
What are Langerhans cells
dendritic cells of the skin/mucosa
146
What are Birbeck Granules
"Tennis-racket" organelle
147
Where is Unisystem Langerhans cell Histiocytosis MCly seen osseously
calvaria, ribs, femur
148
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
Langerhans cell histiocytosis
149
Which condition: systemic coagulation leading to widespread thrombi (lethal), widespread hemorrhage, Tx. anticoags or coags (directed by features)
Disseminated Intravascular Coagulation (DIC)
150
What can the dec. platelets in Thrombocytopenia lead to
Bleeding tendency
151
Which condition: Antibodies (IgG) attack platelets, possible CNS hemorrhage (rare), Tx splenectomy 65% remission
Immune Thrombocytopenic Purpura
152
Which ITP is seen in children, self-limited, secondary to viral infxn
Acute Immune Thrombocytopenic Purpura
153
Which ITP is insidious, ADRs, idiopathic, reproductive age females, easy bruising
Chronic Immune Thrombocytopenic Purpura
154
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)
von Willebrand Disease
155
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
Hemophilia A
156
Which condition: aka Christmas diease, X-linked, 1/20,000 males, Mutated factor IX severe def, Tx. infusion of coag factor IX
Hemophilia B
157
Which condition: Reactive B cells in the thymus, Myasthenia Gravis, SLE, RA, Thymectomy is an early Tx option
Thymic Hyperplasia
158
Which condition: Rare, MC affects adult or MG patients, Benign (encapsulated) or malignant, Asian, Hx. of EBV infxn, Carcinoma obstructive/invasive
Thymoma
