Chapter 20 Flashcards
-hemoglobin synthesis requires ______, ______, ______, and ______
-the red cell production is regulated by the ______ of the arterial blood. a decrease oxygen supply stimulates ______
It is mediated by the kidneys which produce ______
iron vitamin B12 vitamin B6 folic acid oxygen content erythropoiesis erythropoietin
the volume of packed RBC expressed as a percentage of total peripheral blood
hematocrit
denotes the mean volume of RBC
mean corpuscular volume (MVC)
denotes the content of hemoglobin per RBC
mean corpuscular hemoglobin (MHC)
denotes the concentration of hemoglobin in RBC
mean corpuscular hemoglobin concentration (MCHC)
-______is a reduction of hemoglobin in the blood to below-normal levels. Hb is <13.5 g/dL in males and <12.5 g/dL in females. when Hb levels are below ______ then blood transfusion is needed
anemia
8 g/dL
Chronic
inflammation and malignant condition
Anemia due to chronic disease
Microcytic anemia are due to decreased production of hemoglobin (Fe, Hb & protoporphyrin ring structure). Example: Iron deficiency anemia, anemia due to chronic disease, sideroblastic anemia & thalassemia
-RBC size decreases, and lose their color
Microcytic homochromic Anemia
-iron deficiency in anemia: ______
-infants: ______
-children: ______
adults (20-50 years): ______ in males and ______ in females
-elderly: ______ in the western world; ______ infection in the developing world
-other causes include malnutrition (vegetarians), and gastrectomy surgery (acids aid iron absorption by maintaining the Fe2+ state, which is more absorbed than Fe3+)
-serum iron and ferritin levels decrease but total iron binding capacity increase (Transferrin)-Lab test
-supplement of iron (ferrous state Fe2+ high affinity for oxygen)
dietary lack or blood loss breast-feeding (human milk is low in iron) poor diet peptic ulcers menorrhagia or pregnancy colon polyps/carcinoma hookworm
- two forms: idiopathic(no one knows the reason) and secondary
- bone marrow depleted of ______ and consist only of ______, fat cells, and ______
- uncontrollable infections. bleeding tendency, chronic fatigue, sleepiness, and weakness. Patients often crave for ice cubes
aplastic anemia
hematopoietic cells
fibroblasts
scattered lymphpcytes
- caused by deficiency of vitamin B12 or folic acid
- ______: dietary folate is obtained from geen vegetables and some fruits. it is absorbed in the jejunum. (important for hemoglobin production)
- inadequate intake in the diet or because of malabsorption caused by intestinal disease like in alcoholics, elderly or increased demand like pregnancy or cancer
- vitamin B12 deficiency
- ______: autoimmune disease due to autoantibodies against parietal cells and intrinsic factor. common cause of deficiency of vitamin B12
- lack of the ______: stomach cancer or gastric bypass surgery
- diagnosis: ______: by oral radioactive B12 with or followed by cold B12
macrocytic (megaloblastic) anemia folic acid deficiency pernicious anemia gastric intrinsic factors Schilling test
- fatigue, shortness of breath, weakness and neurologic symptoms
- with appropriate and prompt therapy neurological may be reversible
megaloblastic anemia (B12 deficiency)
- genetic defect in the synthesis of HbA that reduces the rate of globin chain synthesis
- no abnormal ______
- ______: reduced synthesis of the alpha chain of globin
- a-thalassemia is usually due to gene deletion; normally, ______ genes are present on ______
- one gene deleted: ______
- two genes deleted: mild anemia with ______ RBC count
- three genes deleted: ______
- four genes deleted: lethal in utero (______). The gamma chains from tetramers (Hb Barts) that damage RBCs. Mothers carrying a fetus with Hb bart have a increased risk for obstetric complications
Thalassemia hemoglobin produced a-Thalassemia 4 alpha chromosome 16 asymptomatic increased severe anemia hydrops fetalis
- reduced synthesis of the beta chain of globin
- ______ or thalassemia trait
- heterozygotes
- mild, nonspecific symptoms
- ______
- homozygotes
- severe and serious disease
- chronic transfusions are often necessary for survival
B-thalassemia
B-thalassemia minor
B-thalassemia major
B-thalassemia patients have ______, facial bones and cranial bones in a characteristic in ______ appearance
distorted long bones
crew-cut
- antibody-mediated (______ or ______) destruction of RBCs
- IgG mediated disease usually involves ______. IgG binds RBCs in the relatively ______ temperature of the central body (warm agglutinin); membrane of antibody coated RBC is consumed by ______, resulting in spherocytes
- associated with ______ (most common cause), and certain drugs (classically, penicillin and cephalosporins)
- treatment involves stoppages of the drugs, steroids, if necessary, ______
- IgM-mediated disease usually involves ______. IgM binds RBCs and fixes complement in the relatively ______ temperature of the extremities (cold agglutinin)
- associated with ______ and ______
immune hemolytic anemia IgG or IgM extravascular hemolysis warm splenic macrophages SLE splenectomy intravascular hemolysis cold mycoplasma infectious mononucleosis
- clonal proliferation of hematopoietic stem cells
- uncontrolled production of red blood cells to mutation. ______ level is decreased
- clinical symptoms are mostly due to ______ of blood. blurry vision and headache, increased risk of ______, flushed face due to congestion (plethora) and itching, especially after ______ (due to histamine release from increased mast cells)
primary polycythemia, or polycythemia vera erythropoietin hyperviscosity venous thrombosis bathing
- increased red blood cell volume owing to bone marrow hyperplasia caused by erythropoietin (increase)
- usually caused by prolonged ______
- living at high altitudes, anoxia secondary to chronic lung disease, congenital heart disease
secondary polycythemia
hypoxia
- reduction in white blood cell count to below-normal levels
- ______: decreased number of circulating neutrophils
- bacterial infections and drug toxicity
- ______: deceased number of circulating lymphocytes
- bacterial, viral, fungal, and parasitic infections
- example: SLE, HIV, etc.
leukopenia
neutropenia (agranulocytosis)
lymphopenia
- increased number of white blood cells in the peripheral blood
- ______: bacterial infection (beginning of the infection)
- ______: allergies and some skin diseases or parasitic infections
- ______: viral infections, chronic infections, some autoimmune disorders
leukocytosis
granulocytosis, or neutrophilia
eosinophilic leukocytosis, or eosiniphilia
lymphocytosis
malignant diseases involving white blood cell precursors in the bone marrow and peripheral blood (acute and chronic)
- myeloid
- lymphoid
malignant diseases of white blood cells
leukemias
lymphoid cell malignant diseases predominantly involving the lymph nodes
- non-hodgkin’s lymphoma
- hodgkin’s lymphoma
malignant diseases of white blood cells
lymphomas
malignant disease of plasma cells
malignant disease of white blood cells
multiple myeloma
- the causes of most lymphomas and leukemias, like the causes of most other malignant tumors, are unknown
- viruses: HTLV-1, EBV
- endogenous oncogenes
- ______ (Philadelphia chromosome)
chronic myelogenous leukemia
- bone marrow is infiltrated with malignant cells
- ______ of blasts; defined as the accumulation of > ______ blasts in the bone marrow
- increased blasts “crowd-out” normal hematopoiesis, resulting in an ______ (fatigue), thrombocytopenia (bleeding), or neutropenia (infection).
- blasts usually enter the blood stream, resulting in a high ______ count
leukemia neoplastic proliferation 20% acute anemia WBC
- most common form of leukemia in children
- massive infiltration of the ______ and ______ with immature lymphoid cells (blasts)
- recurrent infections, generalized weakness, and bleeding into the skin and major internal organs
- treatment: excellent prognosis with ______ (requires prophylaxis to scrotum and CSF)
acute lymphoblastic leukemia
bone marrow
peripheral blood
chemotherapy
- this is the most common form of acute leukemia in adults (50-60 years)
- there is ______ of myeloblasts in the bone marrow (20% myeloblasts)
- without treatment, most patients die within 6 months after the onset of symptoms
- treatment: ______ (vitamin A derivative) for M3 with t(15:17) chromosome
acute myelogenous leukemia
clonal proliferation
all trans retinoic acid
- malignant disease of pluripotent hematopoietic stem cells
- bone marrow and peripheral blood overgrown with ______. associated with Philadelphia chromosome t(9:22)
- treatment: ______ (e.g., imantinib), bone marrow transplant and chemo
chronic myelogenous leukemia
malignant stem cells
tyrosine kinase inhibitors
- ______ of mature B cells characterized by hairy cytoplasmic processes. cells are positive for tartrate-resistant acid phosphatase (TRAP)
- clinical features include ______ (due to accumulation of hairy cells in red pulp area)
- excellent response to ______, an adenosine deaminase inhibitor (adenosine accumulates to toxic levels in neoplastic B cells)
hairy cell leukemia
neoplastic proliferation
splenomegaly
2-CDA (chemo)
- malignant disease involving lymphoid cells
- involvement of lymph nodes leads to generalized ______ and is called ______
- most patients survive 7 to 9 years from the time of diagnosis
treatment: ______
chronic lymphocytic leukemia
lymphadenopathy
small lymphocytic lymphoma
chemotherapy
-it affects any age group
-malignant cells often infiltrate the lymph nodes, spleen, thymus, or bone marrow, but they may also involve any other organ in the body.
extranodal spread of lymphoma
-all age groups are affected (but more common in adults than in children)
lymphoma
- this is the most common form of lymphoma in the US (45% of all)
- it is mostly seen in older people
- the tumor is slow growing
- most patients present with long-standing enlargement of the lymph nodes and only mild constitutional symptoms
- most patients survive 7 to 9 years after diagnosis of the disease
NHL: follicular lymphoma
-this is the most common aggressive form of NHL
-tissues is infiltrated with______ that have irregular nuclear outlines and prominent nucleoli. clinically aggressive (high-grade)
Treatment: ______
NHL: diffuse large-cell lymphomas
large lymphoid cells
chemotherapy
- this is a highly malignant tumor composed of small B cells associated with EBV
- ______ are often more prominent than enlarged lymph nodes
- sub-saharan africa (involves jaw)
- children infected with EBV
- ______: children and young adults most often affected and abdominal mass (e.g., ovarian or intestinal mass). Most children and young adults can be cured
- lab test: characterized by high ______ and ______ appearance on microscopy
burkitt's lymphoma t(8:14) extranodal masses sporadic variant mitotic index starry sky
- EBV infection that results in a lymphocytic leukocytosis comprised of reactive CD8+ T cells.
- EBV is transmitted by ______ (kissing disease) classically affects teenagers and also EBV infects infants
- clinical symptoms: oropharynx, resulting in ______. Liver, resulting in ______ with hepatomegaly and elevated liver enzymes
- diagnostic test: Monotest: detects ______
infectious mononucleosis (IM) saliva pharyngitis hepatitis IgM
- age distribution curve is bimodal, with one peak at 25 years and another at 55 years
- there are 4 types of this disease: (1-4)
- ______ cells are present (hallmark pathological symptom)
- bilobed or multilobed nucleus and prominent nucleoli surrounded by a ______
hodgkin's lymphoma nodular sclerosis lymphocyte-rich mixed cellularity lymphocyte-depleted reed-sternberg clear halo
swollen lymph nodes int he neck (right) sometimes indicate the presence of ______
hodgkins disease
- lymph nodes are ______ (neck and mediastinum)
- extranodal involvement and leukemic spread are rare
- prognosis of the disease depends primarily on clinical stage
- stage I and II tumors are associated with an excellent prognosis and a high rate of cure (>90%) with ______
- advanced disease has a less favorable prognosis
hodgkin’s lymphoma
enlarged
chemotherapy
- malignant disease of plasma cells
- most patients older than 45 years of age
- malignant plasma cells typically proliferate in the ______ and destroy the surrounding bone (bone fractures)
- punched-out holes (______) in the skull, ribs and the vertebrae
- hypercalcemia
- renal failure and ______
- infection
- rouleaux formation of RBCs on blood smear: increased serum protein ______ charge between RBCs
- diagnosis is based on the following:
- X-ray studies (______)
- urine contains bence jones protein (______)
- bone marrow biopsy (neoplastic plasma cells in increased numbers): fried egg appearance
- most patients die within 3 to 4 years, primarily of ______
multiple myeloma bone marrow lytic lesions proteinuria decreases lytic lesions IgG kidney failure or infection
only drug approved to treat multiple myeloma is ______
ninlaro
- congenital clotting factor defects: ______
- acquired clotting factor deficiencies
- ->inadequate production of clotting factors
- hemophilia
- sex-linked congenital clotting factor deficiency
- ______: deficiency of factor VIII
- ______: deficiency of factor IX
- uncontrollable ______ following trauma
- subcutaneous ______ (collection of blood in the tissues under skin)
clotting factor deficiencies hemophilia hemophilia A hemophilia B bleeding hematomas
