Hematology Flashcards
1
Q
formed elements of blood are generated by active (red) bone marrow in which bones?
A
mainly in FLAT BONES of the axial skeleton (skull, ribs, sternum, pelvic bones) and major long bones (femur and humerus)
2
Q
what are the supporting/nourishing adipose connective tissue for active marrow called?
A
yellow marrow
3
Q
what is the process of blood cell and cellular fragments formation called?
A
hemopoiesis or hematopoiesis
4
Q
what is the degeneration of active red bone marrow with substitution by yellow (inactive) marrow called?
A
myelophthisis
seen in debilitating disorders such as blood/bone marrow cancers
5
Q
why are RBCs called formed elements?
A
bc it includes platelets, which are not cells, they are fragments of megakaryocytes
6
Q
what regulates differentiation and growth of progenitor cells?
A
Hematopoietic Growth Factor
7
Q
what is specialized connective tissue composed of liquid matrix called PLASMA that dissolves and suspends various cells and fragments?
A
BLOOD
8
Q
what is part of plasma after removal of clotting factors?
A
serum
9
Q
what hormones are supportive of hematopoiesis?
A
Erythropoietin (EPO) - KD
Thromopoietin (TPO) - LV
Androgens (DHT or ative form of testosterone)
Colongy-stiulating factors (CSFs)
10
Q
which hormone supports hematopoiesis by increasing the # of RBC precursors?
A
Erythropoietin (EPO) from Kidneys
11
Q
which hormone supports hematopoiesis by stimulating the formation of platelets.
A
Thrombopoietin (TPO) from Liver
*TPO mRNA is expressed mainly in LR, and to a lesser extent in KD, SP, LU, bone marrow and brain
12
Q
which hormone supports hematopoiesis by stimulating the production of erythrocytes (RBCs)? This explains a higher ______ in males versus females?
A
Androgens (DHT or active form of testosterone)
it explains higher HEMATOCRIT in Males
13
Q
which hormone supports hematopoiesis by stimulating WBC formation?
A
colony-stimulating factors (CSFs) ir interleukins
14
Q
what are the major fx of blood as tissue?
A
- carrying oxygen and carbon dioxide
- maintaining acid-base balance (buffering) and enabling metabolic reactions
- elimination of waste
- delivery of nutrients
- distribution of hormones and other chemical messengers
- clotting and prevention of excessive bleeding
- immune defense
15
Q
what is fx of the protein albumins in plasma of blood?
A
maintain colloidal osmotic pressure and is a carrier molecule
16
Q
what is fx of the protein fibrinogen in plasma of blood?
A
formation of blood clots, Liver
17
Q
what is fx of the protein globulins in plasma of blood?
A
immunity
18
Q
what is the process of red blood cell formation called?
A
erythropoiesis
19
Q
what are the 3 characteristic trends of erythropoiesis?
A
- w normal DNA, cell is large then gets progressively smaller
- progressive loss of organelles
- increase in cytoplasmic Hemoglobin (Hb) concentration
20
Q
RBCs transport oxygen from LU to the tissues as?
and returns CO2 (volatile acid) to LU as?
A
- oxy-hemoglobin
- carbamino-hemoglobin
21
Q
the packed volume of RBC’s (or all blood cells) is called?
A
Hematocrit (Hct)
[ex: *the solid vegetables in a vegetable soup]
22
Q
what is the normal Hematocrit value in females? in males?
A
F: 37 - 46 %
M: 41 - 53% - due to active testosterone influence
23
Q
what happens to aged (apoptotic) RBCs?
A
they are engulfed by macrophages within the blood, bone marrow, SP, and LR, and digested
24
Q
what happens to the Iron and Hemoglobin of apoptotic RBC?
A
recycled.
- Iron is transported by TRANSFERRINE to tissues, in the form of FERRITIN (mostly in LR)
- Heme is metabolized into bilirubin, and eventually excreted w bile and urine
25
Neutrophils (PMNs) make up what percentage of WBC and fx?
make up 60-75% of all WBC
| fx: primary phagocytes in acute bacteria infections and acute necrosis
26
eosionphils make up what percentage of WBC and fx?
1-3%
| if more abundant in blood signals parasitic infections and allergies
27
basophils fx?
their granules contain histamine, serotonin, heparin, and Slow Reacting Substance of Anaphylaxis (SRS-A), released in certain immune reactions (allergies) causing vasodilation, broncho- , bronchiolo - constriction, and increased permeability of blood vessels, contributing to edema
28
2 types of agranulocytes are?
1. Lymphocytes (specific immune cells, orchestrating all cellular and humoral immune defense events)
2. Monocytes (non-specific immune cells, mainly scavengers
29
3 granulocytes are?
1. neutrophils - (burns, necrosis, stress, pregnancy, strenuous exercise)
2. eosinophils
3. basophils
30
from Myeloid stem cells you get which formed elements?
- RBC (erythrocytes), platelets (thrombocytes)
- granulocytes (eosinophils, basophils, neutrophils)
- monocytes (agranular leukocyte)
31
from Lymphoid stem cells, you get which formed elements?
- T lymphocytes (agranular leukocyte)
| - B lymphocytes (agranular leukocyte)
32
all granulocytes (eosinophils, basophils, and neutrophils/PMNs) and monocytes have a common intermediate precursor, which is?
myeloid precursor
33
malignancies, derived from immature cells of myeloid origin are called?
myeloid or myelogenous (myelocytic) leukemias
34
malignancies derived from lymphoid progenitor/precursors are called?
lymphomas or lymphocytic/lymohoblastic leukemias
35
what are the dual meaning of myeloid?
1. another name for active bone marrow
| 2. malignancies, derived from immature cells of myeloid origin
36
what is immediate precursor cell for mature erythrocyte?
reticulocyte (immature RBC)
37
what is immediate precursor cell for mature neutrophil
band cell
38
what is immediate precursor cell for multiple fragments, called thrombocytes (platelets)?
megakaryocyte
39
very young/immature formed elements/cells of blood are usually addressed as ?
"blasts"
ex: megakaryoblast --> megakaryocytes --> platelets
ex: eosinophillic myeloblast --> eosinophil
40
increased WBC count indicates?
and unless state otherwise, this should be understood as increase of what?
Leukocytosis
increase in Neutrophil,
[and suspect acute bacterial infections first, followed by acute inflammation necrosis, pregnancy, stress]
41
decreased WBC indicates?
due to?
Leukocytopenia
[immunodeficiency, predisposing to overwhelming bacterial (pyogenic) infections
42
increased in eosinophil count indicates?
due to?
eosinophilia
parasitic infections, allergies
43
basophilia indicates?
allergies
44
increase in cells have suffix?
- sis
| ex: lymphocytosis, leukocytosis
45
decrease in cells have suffix?
- penia
| ex: lymphocytopenia, leukocytopenia
46
which condition is transient (episodic) and will most commonly signify viral infection, and if it is persistent should be evaluated for auto-immume pathologies, or if cells are immature, for WBC cell malignancies?
lymphocytosis
47
which condition is usually associated with immunodeficiency, esp when T lymphocytes is deficient?
lymphocytopenia
48
which condition has deficient count of RBC and or hemoglobin concentration?
anemia
[in aplastic anemia - all blood cells are extremely def count]
49
what condition indicates anemia or hypoxia - suspect chronic bleeding, respiratory pathology, smoking, change of altitude, convalescence stage (recovery)?
and has an increase of young/immature RBC from peripheral circulation?
reticulocytosis
| increase in reticulocyte count
50
what condition has an increase in immediate precursor cells for neutrophils and suggests initial stage of acute bacterial infection?
bandemia
51
what is an increase in mainly RBC count (increase in hematorcrit, the formed elements, and NOT the volume of blood)
polycythemia
52
what condition of polycythemia is due to diminished plasma volume (dehydration by profuse diaphoresis, emesis, diarrhea, heat exhaustion, burns)?
relative polycythemia
53
Absolute Polycythemia is divided into two subgroups what are they and what does it mean?
1. Primary absolute polycythemia / polycythemia rubra vera - bone marrow malignancy as myelo-proliferatice disorder - bone marrow is not well/cancerous and overproduction of RBC makes blood like stew. RBC are overcrowded causing injury to themselves and to walls of endothelium, attracting platelets causing thrombosis and stagnation.
2. Secondary absolute polycythemia - renal cell carcinoma with over production of erythopoietin, or extreme need for erythropoiesis (due to smoking, chronic CO or CO2 poisoning)
54
what is MCV?
mean corpuscular volume 80 - 95 femtoliter
size of cell
[small cells usu hemoglobin problem
large cells usu DNA def]
55
what is MCH?
mean corpuscular hemoglobin 27 to 31 pg/cell
56
what is RDW?
Red Cell Distribution Width 11/5 - 14.5
57
what is MCHC?
mean corpuscular hemoglobin concentration
32 to 36 gm/dL
if MCHC is low - cell is hypochromic
if high, hyperchromic
[usu small cells have hemoglobin problem]
58
anemia can result from one or more of 3 basic mechanisms, what are they?
1. blood loss
2. def erthropoiesis (due to iron def, folate def, B12/cyanocobalamin def, copper def, chronic debilitating illness)
3. excessive hemolysis (due to sickle cell anemia, thalassemia, malaria, auto-immune etc)
59
which morphological classification of anemia is characterized by smaller cell size (diminished MCV) and poor HB content (pale cells), usually due to deficient Hemoglobin production?
microcytic and hypochromic anemia
if iron def - microcytic MCV is low, and hypochromic MCHC is low
60
1. The major and most common microcytic anemia is due to what?
2. And the major etiology of this condition is?
3. what is MCV and MCHC like?
1. IRON DEF
2. chronic (insidious) blood loss???
3. if iron def - microcytic MCV is low, and hypochromic MCHC is low
[MCV is less than 80 femtoliter]
61
1. which morphological classification of anemia is usually due to deficient DNA production, which contributes to inability of RBCs to mature, so they are arrested in a larger size with increased MCV?
2. what is the most common etiology of this condition?
1. macrocytic --> megaloblastic
| 2. deficiency of B12 or Folate content
62
which morphological classification of anemia can be due to chronic disease or an acute blood loss?
normocytic and normochromic anemia
63
Iron Def anemia presents which what important clinical characteristics?
what are CBC test results likely to show?
pica (craving dirt), pagophgia (ice), koilonychia (brittle nails), glossy tongue
RBC appear pale (hypchromic) and small (microcytic) - low MCV less than 80; and low MCHC less than 32
64
Hemolytic anemia of any origin will show?
Prominent JAUNDICE!!
65
anemia of chronic disease will show
normal RBCs count, but diminished Hematocrit
66
what is TIBC?
Total Iron Binding Capacity
67
The TIBC In iron def anemia is increased or decresed?
increased, the capacity to bind iron to transferrin is increased as the receptors on the transferrin are less saturated
68
the TIBC in hemolytic anemia is increased or decresed?
decreased, transferrin sites will be oversaturated with iron
69
What are the 2 most common type of macrocytic or megaloblastic anemia?
between the 2 types, which is more common and why?
Folic Acid (B9) Def Anemia and B12 (Cyanocobalmin) Def Anemia
Folic acid is more common than B12 def, due to inability of human body to store folate
70
what is fx of B12 in body?
Vitamin B 12 is important for healthy status of nervous and cardio-vascular systems. Chronic B12 def has strong correlation with irreversible neurological deficits (severe neuropathies due to demyelination within the CNS) and ischemic heart disease
71
what is a well known cause of Spina Bifida syndrome and what is this?
folate def during pregnancy causes this.
| this is developmental congenital disorder causes by incomplete closing of embryonic neural tube
72
what is pernicious anemia, and why is it called pernicious?
patient who suffer chronic auto-immune atrophic gastritis are unable to produce intrinsic factor complex as carrier molecules for cyanocobalamin (B12) absorption through the mucosal barrier of jejuno-ileum.
this complex is produced by parietal cells of ST, which are atrophied in this condition.
it's pernicious bc, this is prerequisite to gastric cancer
73
food sources for B9 (folate) and B12
B 9 - leafy green vegetables
| B12 animal sources
74
what is Sickle Cell Anemia?
hereditary hemolytic anemia, which is caused by point mutation on gene, coding for beta-chain of adult Hemoglobin A
it is of an autosomal-recessive inheritance mode (need 2 genes; from mother and father)
- as a result, glutamic acid is substituted by valine at position 6 of beta-chain in Hemoglobin molecules, leading to production of Hemoglobin S instead of Hemoglobin A.
75
IN SC anemia, hemoglobin S is prone to crystallization under what conditions?
hypoxia, acidosis, stress, dehydration - which in turn leads to sickling of the RBCs in peripheral circulation
76
what is sickling of cells in SC anemia?
RBC becomes rigid and becomes "ice pick" shape --> RBC keeps stalking in the microcirculation, and trigger injury to selves and endothelium of blood vessels --> promotes thrombosis --> leads to ischemia
77
autosplenectomy in SC anemia happens how? and can lead to?
- early in childhood spleen may be enlarged due to continual stasis and trapping of abnormal RBC's leads to infarctions that eventually reduce the size of spleen tremendously by adolescence.
- autosplenectomy leads to severe immuno-def
78
what is frontal bossing in SC anemia, and why does it occur?
severe, chronic anemias (such as SC anemia) can increase bone marrow response to form RBC --> this drive for erythropoiesis may increase the mass of marrow and lead to abnormal increase in marrow places -- "frontal bossing" --> eventually, this causes brain injury by increased intracranial pressure and brain herniation
-
79
what are clinical manifestation of SC anemia?
hemiparesis, severe headache, dysphasia
splenomegaly, drop in hemoglobin
hematuria, proteinuria, painful sustained erection
abd pain, hepatomegaly
pain, decreased in vision, blindness
pulmonary infection, chest pain, cough, dyspnea
swollen hands and feet pain, decreased mobility
80
what is sustained, painful, and unwanted erection of the penis, which is a result of either increased arterial inflow or failure of venous outflow called? it is serious complication of what disease?
and what can it lead to?
priapism - serious compliction of SC anemia
if sustained for more than 3 hrs, necrosis of penis tissue
81
what is Aplastic Anemia and why is this s misnomer?
tx?
etiology?
pancytopenia (total def of all lines of blood cells)
failure of bone marrow to generate any lines of blood cells
clinical presentation very similar to leukemia, may need bone marrow transplantation
misnomer bc it doesn't deal solely with RBC
etiology is unknown but suspect chemical and drug toxicity, irradiation, hereditary, and advanced stages of blood cancers
82
what are common suspected etiologies of Leukemias?
genetic factors, ionizing radiation, chemotherapy drugs, viruses, endogenous oncogenes
83
what account for more than 30% of all leukemias and about 80% of childhood leukemia?
does it respond well to tx?
Acute Lymphocytic or lymphoblastic leukemia (ALL)
| *respionds well to tx
84
which leukemia derives from immature granulocytes and/or monocytes and accounts for the most common leukemia of adulthood?
young adults are most frequently affected.
what is distinct feature of the myeloblasts in this leukemia?
actue myelogenous/myeloid/myelocytic leukemia (AML)
*nuclear victims / like hiroshima, Chernobyl
linear red "Auer rod" composed of crystallized granules
85
which leukemia is slow-progressing within approx a decade in older adults and is poorly responsive to chemotherapy?
and could also be related to leukemic component of non-Hodgkin's lymphoma?
does it respond well to tx?
Chronic Lymphocytic leukemia (CLL)
*responds poorly to tx
86
which leukemia is most strongly associated with so called Philadelphia chromosome (a translocation of genes bt chromosomes 9 and 22)?
this is most prevalent in what age group?
Chronic Myelogenous Leukemia (CML)
middle-aged adults
87
Chronic or Acute leukemia, which is more indolent because they are based mostly on comparatively more differentiated (less anaplastic) cells?
chronic - more remissions, cells are still immature but more matured than acute leukemias, trigger and contribution factors are chemical poisoning of bone marrow
88
which cancer is monoclonal (originated from one cell) plasma cell?
multiple myeloma (MM)
89
what is plasma cells?
| and what happens with these cells in MM?
plasma cells, which are activated B lymphocytes, are immature in multiple myeloma and spread indefinitely within the bone and peripheral blood.
they secrete abnormal fragments of antibodies (IgG, IgM, and other immunoglobulins), which are not mature and not structured efficiently to ensure humoral immunity.
These abnormal fragments clamp together and produce Bence-Jones proteins, obstructing peripheral circulation and esp KD tubules.
eventually, bone marrow "gives up" and aplastic anemia crisis is an end-outcome of the disease.
90
what is Bence - Jones protein?
pieces of unwind protein - the clump together and clog KD tubules
91
1. why is MM considered blood and bone cancer?
2. tx? and etiology?
3. what is end result of MM?
1. bc bone marrow generates crazy fragments
2. tx is difficult, the cancer is fairly aggressive, etiology is obscure
3. bone marrow produce clone of one defective cell and end of disease is APLASTIC ANEMIA, before it gets this far though most often patient suffers neurological disorders and die
92
which cancer creates "Moth-eaten" lesions within the skull?
Multiple Myeloma
93
what are the two types of lymphoma and what differentiates them?
Hodgkin's and non-Hodgkin's
-all lymphomas are cancers of lymphatic structure, however, non-H could have simultaneous component of lymphocytic leukemia as well.
H is "solid"
non-H is "solid in liquid"
[thus, testing of peripheral blood is poor dx tool for H]
H - Reed-Sternberg bi-nucleated cells found within the parenchyma of lymph nodes, SP, LR
non-H - NOT characterized by specific cells
94
which lymphoma has Reed-Sternberg bi nucleated cells?
| what does this cell look like?
Hodgkin's lymphoma
large cells with large, pale nuclei containing large purple nucleoli, looks like owl eyes, it has 2 nucleoli within the cell
they contain reactive lymohoid cells
95
what is the major definitive dx of lymphoma?
by needle or surgical biopsy of lymphatic structures and bone marrow biopsy
96
Hodgkin's lymphoma is characterizied by what types of lymph nodes?
usually painless lymph nodes, but if it is less than 1 cm in size, and is soft and pink, have it biopsied bc alcohol can make this node painful.
97
there are many different types of non-Hodgkin's Lymphoma, what is one type we learned in class?
and is associated with which infection?
Burkitt's lymphoma
associated w epstein barr infection
98
what is def of platelets?
| and this is defined by platelet count of less than ________?
thrombocytopenia
less than 100,000 cell/mcl of blood
99
what are causes of thrombocytopenia?
1. decreased platelet production by bone marrow as in aplastic anemia
2. increased platelet removal from the peripheral circulation as with enlarged SP (the SP is "blood cell recycling factory")
3. overconsumption or an injury of platelets in severe generalized bacterial infections (sepsis) or auto-immune reactions, mediated by toxins, viruses, drugs, venomous bites
100
what is hemophilia?
who is affected and why?
inefficient coagulation cascade (not enough "dancing and knitting" of glycoproteins)
Hemophilia A and B are both hereditary X-linked recessive disorders of predominately males. THey are caused by mutations of the genes, which code for clotting factors VIII and IX respectively.
[females are usu carriers, and Males are affected bc they only have one X chromosomes
(possible for F to suffer in case of incestuous relations, etc)]
101
what is the most common type of hemophilia?
Hemophilia A (def of clotting factor VIII
102
which hemophilia is also known as Christmas disease by teh name of hte first patient described and which clotting factor is def?
Hemophilia B
def of clotting factor IX
103
what is the most common genetic bleeding disorder and what are its characteristics?
Von Wilebrand Disease - caused by genetic defect of different inheritance mode (mostly autosomal) of gene, coding for von Wilebrand factor production.
-very similar to hemophilia in its clinical presentation, however, has more relevance of heavy menstrual bleedings along with spontaneous bleeding and thrombosis. It should be differentiated w other bleeding disorders by essaying certain clotting factors.
