blood and lymph WTFSDKJAFVHAJWEHFBJAEFAFV Flashcards

1
Q

Hematopoietic System

A

Erythrocytes ideally suited for primary function: transport of oxygen from lungs into peripheral tissues

Hemoglobin: complex molecule consisting of four heme groups & four globins

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2
Q

A

Objective measurements of red blood cell parameters: done with instruments estimating mean size of red blood cells & their hemoglobin content
Mean corpuscular volume (MCV)
Mean corpuscular hemoglobin (MCH)
Mean corpuscular hemoglobin concentration (MCHC)
White blood cells participate in body’s defense against infections
Neutrophils: short-lived cells that survive no more than 4 days in peripheral circulation
Monocytes & lymphocytes: long-lived blood cells

Platelets or thrombocytes: essential clotting factors
Malignant transformation of hematopoietic cells may result in solid tumors or leukemia

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3
Q

Anemia

A

Reduction of hemoglobin in blood to below normal levels

In practice, this means

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4
Q

Anemia may be a consequence of:

A

Decreased hematopoiesis
Abnormal hematopoiesis
Increased loss or destruction of red blood cells

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5
Q

Decreased Hematopoiesis

A

Bone marrow failure

  • -Aplastic anemia
  • -Myelophthisic anemia

Deficiencies of nutrients

  • -Deficiency of vitamin B12 & folic acid (megaloblastic anemia)
  • -Protein deficiency
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6
Q

Abnormal Hematopoiesis

A
  • Usually a consequence of genetic abnormalities

- Sickle cell anemia

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7
Q

Increased Loss & Destruction of Red Blood Cells

A

Bleeding
Intrasplenic sequestration
Immune hemolysis
Infections (malaria)

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8
Q

Morphology of Anemias

A

Normocytic, normochromic anemia
-“Dilutional anemia”
Microcytic, hypochromic anemia
-Iron deficiency
Macrocytic, normochromic anemia
-Deficiency of vitamin B12 and/or folic acid
Anemias characterized by abnormal red blood shapes
-Elliptocytosis, spherocytosis, sickle cell

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9
Q

Aplastic Anemia

A

Idiopathic, secondary
Bone marrow depleted of hematopoietic cells; consists only of fibroblasts, fat cells, scattered lymphocytes
Anemia, leukopenia, thrombocytopenia
Uncontrollable infections, bleeding tendency, chronic fatigue, sleepiness, weakness

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10
Q

Thrombocytopenia

A

Deficiency of platelets in the blood.

This causes bleeding into the tissues, bruising, and slow blood clotting after injury.

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11
Q

Iron Deficiency Anemia

A
Most common form of anemia
Hypochromic, microcytic anemia
Etiology:
Increased loss of iron (e.g., chronic bleeding)
Inadequate iron intake or absorption
Increased iron requirements
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12
Q

Megaloblastic Anemia

A
Caused by deficiency of vitamin B12 or folic acid
Vitamin B12 deficiency 
-Pernicious anemia
-Lack of gastric intrinsic factor
-Atrophic gastritis
Folic acid deficiency
--Inadequate intake in diet or malabsorption caused by -=intestinal disease
Bone marrow
-Hypercellular, numerous megaloblasts
Peripheral blood
-Macrocytic anemia
Hypersegmentation of neutrophils

Fatigue, shortness of breath, weakness

Destruction of posterior & lateral columns in spinal cord:
  results in loss of sense of vibration, proprioception, & loss of deep tendon reflexes

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13
Q

Hemolytic Anemia

A

-Increased red blood cell destruction (hemolysis)
-Intracorpuscular defects
Structural abnormalities
Sickle cell anemia, thalassemia, hereditary spherocytosis
-Extracorpuscular defects
Antibodies, infectious agents, mechanical factors
Autoimmune hemolytic anemia, hemolytic disease of newborn, transfusion reactions, malaria, hemolytic anemia caused by cardiac valve prosthesis, disseminated intravascular coagulation
–Anemia (i.e., low erythrocyte count)
-Compensatory erythroid hyperplasia of bone marrow
-Hyperbilirubinemia, jaundice

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14
Q

Sickle Cell Anemia

A

Substitution of glutamic acid by valine
Synthesis of abnormal beta chain of globin
Most prevalent among African Americans
Multiple infarcts in various organs
Neurologic defects; sharp pain in bones, spleen (autosplenectomy), extremities; retinal infarcts
Hyperbilirubinemia, jaundice (bile stones)

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15
Q

Sickle Cell Anemia:Long-Term Complications

A

Retarded intellectual development, neurologic deficits
Cardiopulmonary insufficiency
Recurrent infections

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16
Q

Thalassemia

A
  • Genetic defect in synthesis of hemoglobin A (HbA); reduced rate of globin chain synthesis
  • No abnormal hemoglobin produced
  • Beta-thalassemia: reduced synthesis of beta chain of globin
  • Alpha-thalassemia: reduced synthesis of alpha chain of globin
---Thalassemia minor, or thalassemia trait 
Heterozygotes
Mild, nonspecific symptoms
---Thalassemia major 
Homozygotes
Severe, serious disease

Hyperbilirubinemia, jaundice
Chronic anemia that retards growth of children
Impairment of normal intellectual development
Cardiorespiratory insufficiency

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17
Q

*Hemosiderosis(US) or haemosiderosis (Br)

A

is a form of iron overload disorder resulting in the accumulation of hemosiderin. Types include: Transfusionhemosiderosis. Idiopathic pulmonaryhemosiderosis.

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18
Q

Immune Hemolytic Anemias

A
Mediated by antibodies that destroy red blood cells
(Autoantigens
Alloantigens
Neoantigens)
Mismatched blood transfusion
Hemolytic disease of newborn
Autoimmune hemolytic anemias
19
Q

Polycythemia

A

Erythrocytosis

Increased number of red blood cells

20
Q

Primary polycythemia, or polycythemia vera

A

Myeloproliferative disorder
Clonal proliferation of hematopoietic stem cells
Uncontrolled production of red blood cells, increased total red blood cell mass

21
Q

Secondary polycythemia

A

Increased red blood cell volume as a result of erythroid bone marrow hyperplasia caused by erythropoietin
Usually caused by prolonged hypoxia
Risk factors: living at high altitudes, anoxia secondary to chronic lung disease, congenital heart disease, renal carcinoma

22
Q

Polycythemia: Symptoms

A
Hypertension
Dark red or flushed face
Headaches, visual problems, neurologic symptoms
Splenomegaly
Hypercellular bone marrow
23
Q

Leukopenia

A

Reduction in white blood cell count to below normal levels
Neutropenia (agranulocytosis)
Bacterial infections
Lymphopenia
Bacterial, viral, fungal, parasitic infections

24
Q

Leukocytosis

A

Increased number of white blood cells in peripheral blood
Granulocytosis (neutrophilia)
Bacterial infection
Eosinophilic leukocytosis (eosinophilia)
Allergies, some skin diseases or parasitic infections
Lymphocytosis
Viral infections, chronic infections, some autoimmune disorders
Splenomegaly, lymphadenopathy

25
Malignant Diseases of White Blood Cells
1.Leukemias: malignant disease involving WBC precursors in bone marrow, peripheral blood (acute & chronic) Myeloid Lymphoid 2.Lymphomas: lymphoid cell malignant diseases predominantly involving lymph nodes Non-Hodgkin’s lymphoma Hodgkin’s lymphoma 3.Multiple myeloma: malignant disease of plasma cells
26
Lymphoma: Etiology and Pathogenesis
Causes of most lymphomas, leukemias (like causes of most malignant tumors) unknown Viruses HTLV-1 EBV Endogenous oncogenes t(8,14): Burkitt’s lymphoma t(9,22): chronic myelogenous leukemia (Philadelphia chromosome)
27
Leukemia
Bone marrow: infiltrated with malignant cells Increased number of immature blood cells in peripheral blood Complications: anemia, recurrent infections, uncontrollable bleeding
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Acute Lymphoblastic Leukemia
Most common form of leukemia in children Massive infiltration of bone marrow, peripheral blood with immature lymphoid cells (blasts) Symptoms: recurrent infections; generalized weakness; bleeding into skin, major internal organs Treatment: modern chemotherapy; remission possible in nearly all patients
29
Acute Myelogenous Leukemia
Most common form of acute leukemia in adults Clonal proliferation of myeloblasts in bone marrow (20% myeloblasts) According to WHO, four forms of AML: AML with recurrent genetic abnormalities AML evolving from multilineage dysplasia Therapy-related AML AML not otherwise specified Without treatment, most patients die within 6 months after symptom onset
30
Chronic Myelogenous Leukemia (Cont’d)
-Malignant disease of pluripotent hematopoietic stem cells -Bone marrow, peripheral blood overgrown with malignant stem cells and descendants Three phases of disease: 1,Chronic phase 2.Accelerated phase 3.Blast crisis -Treatment: tyrosine kinase inhibitors (e.g., imatinib, “Gleevec”) Philadelphia (Ph1) chromosome, with BCR/ABL gene rearrangement
31
Chronic Lymphocytic Leukemia
Malignant disease involving lymphoid cells Most patients older than 50 Should be suspected if lymphocytes >5000/mL Median survival: 7–9 years from diagnosis
32
Lymphoma
-Affects any age group -Malignant cells often infiltrate lymph nodes, spleen, thymus, or bone marrow, but may also involve other organs Extranodal spread of lymphoma -Two large categories: Non-Hodgkin’s lymphoma (NHL) Hodgkin’s lymphoma
33
Non-Hodgkin’s Lymphomas
No benign lymphomas Most have B-cell phenotype All age groups affected (more common in adults than children) Can spill over into blood; present as leukemia Involve lymph nodes, bone marrow, spleen, thymus, but can be of extranodal origin Most common sites of extranodal lymphomas: stomach, intestines
34
Non-Hodgkin’s Lymphomas: Diagnosis
--Light microscopy --Ancillary techniques Immunohistochemistry Flow cytometry Cytogenetic analysis ---Clinical features Lymph node enlargement Systemic constitutional symptoms Extranodal tumor spread
35
Follicular Lymphoma
Most common form of lymphoma in U.S. (45% of all) Mostly seen in older people Slow growing Presents with long-standing enlargement of lymph nodes, mild constitutional symptoms Median survival: 7–9 years after diagnosis
36
Diffuse Large-Cell Lymphomas
Most common aggressive form of NHL Tissue infiltrated by large lymphoid cells with irregular nuclear outlines, prominent nucleoli Complete remission induced in 75% of patients with chemotherapy
37
Burkitt’s Lymphoma
Highly malignant tumor composed of small B cells Extranodal masses: more prominent than enlarged lymph nodes Endemic variant: Sub-Saharan Africa Children infected with EBV Mandible, facial soft tissue involvement Sporadic variant: Children, young adults most often affected Abdominal mass (e.g., ovarian or intestinal mass) Most children & young adults can be cured
38
Hodgkin’s Lymphoma
``` Bimodal age distribution curve: peaks at 25 & 55 years 5 types: Nodular sclerosing Lymphocyte predominant Lymphocyte rich Mixed cellularity Lymphocyte depleted ``` Reed-Sternberg cells present Bilobed or multilobed nucleus, prominent nucleoli surrounded by clear halo
39
Hodgkin’s Lymphoma:Clinical Features
Enlarged lymph nodes (neck, mediastinum) Extranodal involvement, leukemic spread: rare Prognosis: depends primarily on clinical stage Stage I & II: excellent prognosis, high rate of cure (>90%) with chemotherapy Advanced disease: less favorable prognosis
40
Multiple Myeloma
Malignant disease of plasma cells Most patients older than 45 years Malignant plasma cells typically proliferate in bone marrow, destroy surrounding bone (bone fractures) Punched-out holes (“lytic lesions”) in calvaria vertebrae Hypercalcemia Renal failure Anemia, leukopenia ``` Diagnosis based on: X-ray studies (lytic lesions) Serum electrophoresis (monoclonal spike) Bone marrow biopsy (neoplastic plasma cells in increased numbers) Survival rate: ```
41
Normal Hemostasis
Vascular factors Platelet factors Coagulation factors
42
Platelet Disorders
Decreased production of platelets - Aplastic anemia - Leukemia - Drugs - Infectious agents Increased intravascular destruction of platelets or increased consumption - -Systemic lupus erythematosus - Various forms of hemolytic anemias - Drug-induced hematologic disorders - Idiopathic thrombocytopenia purpura (ITP) Increased removal of platelets -Hypersplenism Consumption of platelets -Disseminated intravascular coagulation Disorders of platelet function - Congenital disorders (thrombasthenia) - Acquired disorders (chronic renal failure)
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Acquired Clotting Factor Deficiencies
Inadequate production of clotting factors Excessive consumption of clotting factors Action of anticoagulants Hemophilia Sex-linked congenital clotting factor deficiency Hemophilia A: deficiency of factor VIII Hemophilia B: deficiency of factor IX Uncontrollable bleeding after trauma Subcutaneous hematoma or hemarthrosis Prolonged aPPT (prothrombin time normal)
44
Hemophilia
``` Sex-linked congenital clotting factor deficiency Hemophilia A: deficiency of factor VIII Hemophilia B: deficiency of factor IX Uncontrollable bleeding after trauma Subcutaneous hematomas or hemarthrosis Prolonged aPPT (prothrombin time normal) ```