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What is the term for a reduction in the oxygen-transporting capacity of the blood, usually due to reduction of circulating RBC? Anemia
Anemias are classified according to what? Morphology of Red Blood Cells (RBCs)
Mean corpuscular volume MCV (vol/RBC)
Mean corpuscular hemoglobin MCH (Hb/RBC)
MCH concentration Mean corpuscular Hb concentration (Hb/vol), MCH/MCV.
Inc MCV, Inc MHV & normal MCH Macrocytic
Normal MCV, MHV & MCH conc Normocytic
Dec MCV, Dec MHV & normal MCH conc Simple Microcytic
Dec MCV, Dec MCH & Dec MCH conc Hypochromic Microcytic
What are the clinical presentation of Anemia? (3) Pallor, fatigue & lassitude
What is the term for Anemias that are associated with decreased RBC Life Span? Hemolytic Anemia
What is the normal RBC life span? 120 days
Name the two types of Hemolytic Anemia and their differences. Intracorpuscular Hemolytic Anemia (inherited) & Extracorpuscular Hemolytic Anemia (Acquired)
Hemolytic Anemia can produce what 3 types of increases? Increased Hematopoiesis, Increased Phagocytocis & Increased iron in blood and tissue.
What 3 organs are affected with increased Hematopoiesis and their names? Liver (Hepatomegaly), Spleen (Spleenomegaly) & Marrow enlargements.
Increased peripheral circulation of immature RBCs. Reticulocytosis
2 common location for removal of RBC. Liver and Spleen by Macrophage
What happens to iron released into the blood? Converted into Bilirubin in the blood, altered into Conjugate Bilirubin in the liver, and excreted through the Bile in feces.
Excess Bilirubin deposited in tissue is converted by _____ and the term is called _______. Hemosiderin. Termed: Hemosiderosis or Hemochromatosis.
Name the 4 types of hereditary (Intracorpuscular) Hemolytic Anemia 1. Hereditary Spherocytosis 2. Sickle Cell Anemia 3. Thalassemia 4. Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD Deficiency)
What is the inherited Hemolytic Anemia characterized by Normal RBC but spheroidal in shape, less deformable & vulnerable to splenic destruction? Hereditary Spherocytosis
Lysing of RBCs in the SPLEEN due to Hereditary Spherocytosis can result in what extreme condition? Splenomegaly (enlarged spleen)
What is the term for the surgical removal of the spleen? Splenectomy
What is the inherited Hemolytic Anemia characterized by the replacement of normal Hb-A by abnormal Hb-S. Sickle Cell Anemia
What is the gene expression for Sickle Cell Anemia? Codominance
Term for Sickle Cell Anemia where all Hb-A is converted to Hb-S? (Homozygous) Sickle Cell Disease
Term for Sickle Cell Anemia where some of the Hb-A is converted to Hb-S? (Heterozygous, 40%) Sickle Cell Trait
Under what condition does the RBC sickle in Sickle Cell Disease? Normal Oxygen Tension
Under what condition does the RBC sickle in Sickle Cell Trait? Low Oxygen Tension
What are the 3 common factors that influence the sickling of RBCs? 1. Hemoglobin other than Hb-A 2. MCH conc per cell 3. Low oxygen
Term for shrunken, fibrotic spleen with resultant loss of function? (usually due to lysed RBC accumulation in the spleen from Hemolytic Anemia) Autosplenectomy
What is the lifespan of Sickled RBCs? 20 days
What is the anemic classification (morphology) of Sickle Cell Anemia? Normocytic Hemolytic Anemia
Term for Sudden pain in the abdomen, chest and joins due to sickle cell anemia? Sickle Cell Crisis
Term for necrosis of the marrow that lead to a fat emboli that causes Pulmonary ischemia and inflammation? Acute Chest Syndrome
What are the two leading causes of ischemic-related deaths in patients with sickle cell anemia? Acute Chest Syndrome and Stroke
Term for film findings (in bone) with individuals with active hematopoiesis (RBC production). Hair-on-end skull film
What is the name of the Hemolytic Anemia characterized by insufficient synthesis of alpha or beta globin chains? Thalassemia
This autosomal codominant disorder secondary to one of 90 DNA MUTATION that affect beta chains, and common among Mediterranean, African and Asian population. Beta-Thalassemia
What are the two forms of Beta-thalassemia and their gene expression? Thalassemia Minor (Heterozygous) & Thalassemia Major (Homozygous)
What is the Thalassemia that results form GENE Deletion? Alpha-Thalassemia
What is the unique characteristic of Alpha-Thalassemia? Excess accumulation of intracellular alpha chains forms insoluable Aggregates.
RBCs with excess aggregates often "removed" by macrophage that result in what type of cells? Bite Cells
How many possible deletion are there that produce Alpha-thalassemia? Four 1. Carrier state 2/3 Alpha thalassemia 4. lethal in utero.
Term for developing RBCs in the marrow that die by apoptosis? Ineffective Erythropoiesis
What is the anemic classification (morphology) of Homozygous development of Hb-F rather than Hb-A? Hypochromic Microcytic Anemia
What is the inherited Hemolytic Anemia characterized by RBC being vulnerable to oxidents? Glucose-6-phosphate dehydrogenase deficiency (G6PD Deficiency)
Term for inclusions formed by RBC's exposed to oxidents, denaturing the hemoglobin. (typically Normal in size, bite cells seen in smears) Heinz bodies
Type of RBC most affected by G6PD deficiency. Older cells.
Name the 3 causes of Acquired (Extracorpuscular) Hemolytic Anemia. 1. Erythroblastosis Fetalis 2. Mechanical Trauma 3. Malaria
Name of the Hemolytic disease of the Newborn (mother antibodies attacking babies RBC) Erythroblastosis Fetalis
Name the 2 common types of Mechanical Trauma 1. Traumatic Hemolytic Anemia 2. Microangiopathic Hemolytic Anemia
Clinically significant hemolysis of RBC by mechanical damage by artificial cardiac valve that produce abnormal shear stress from alteration of blood flow. Traumatic Hemolytic Anemia
Narrowing or obstruction of vasculature that damage RBC. Microangiopathic Hemolytic Anemia
Peripheral smear of fragmented RBCs (3) Burr cells, Helmet cells, & Triangle Cells
Acquired (Extracorpuscular) Hemolytic Anemia due to organism that invade liver cells and enter RBCs, causing them to Lyse. Malaria
What is the protozoa responsible for malaria? Plasmodium Falciparum
What is the term for hemoglobinuria due to hemolysis from Malaria? Blackwater Fever
3 types of Anemia due to Decreased RBC production (Diminished Erythropoiesis) 1. Nutritional Anemia 2. Aplastic Anemia 3. Myelophthisic Anemia
4 types of Nutritional Anemia 1. Iron Deficiency Anemia 2. Anemia of Chronic Disease 3. Folic Acid Deficiency Anemia 4. Vit B12 Deficiency Anemia
Most prevalent type of Nutritional Anemia and seen predominantly in women in reproductive years. (also seen in adolescent, alcoholics, and elderly) Iron Deficiency Anemia
Most iron is obtained in what form? Heme of animal products
What is the anemic classification (morphology) of Iron Deficiency Anemia? Hyprochromic Microcytic Anemia
What are the clinical manifestation of Iron Deficiency Anemia? Weakness, Listlessness and Pallor
Term for compunction to consume dirt or clay seen in individuals with severe Iron deficiency. Pica
Due to Pica, this may occur which consist of atrophic glossitis, dysphagia, and esophageal web with increased frequency of oral and esophageal carcinoma. Plummer Vinson Syndrome
What is the anemic classification (morphology) of Anemia of Chronic Disease? Normocytic Normochromic or Microcytic Hypochromic
Anemia of Chronic Disease may arise from what 3 causes? 1. Chronic Infection 2. Chronic Immune Disorders 3. Neoplasms
What is the Nutritional Anemia caused by a lack of folate? Folic Acid Deficiency Anemia
What is the anemic classification (morphology) of Folic Acid Deficiency Anemia? Megaloblastic Macrocytic Anemia
How does Folic Acid deficiency contribute to Megaloblastic Macrocytic Anemia? Folic Acid is required for DNA synthesis, deficiency will lead to arrest of development leading to Megaloblastic Macrocytic RBCs.
What is the Nutrition Anemia characterized by inadequate intake or malabsorption of Vit. B12? Vit B12 Deficiency Anemia
What is the difference between Folic Acid and Vit B12 Deficiency? Vit B12 produced neurological abnormalities, of which the most important is demyelination of the spinal cord.
Because dietary lack is rare most examples of Vit B12 Deficiency anemia are? Pernicious Anemia
Pernicious Anemia Characteristic Autoimmune disease against Parietal cells & Intrinsic factors leading to malabsorption of Vit B12.
What type of factor is Vit B12 considered to be? Extrinsic factor
What is the diagnostic test for malabsorption of Vit B12? Schilling Test
What Diminished Erythropoiesis is characterized by suppression of multipotent myeloid stem cells, with resultant anemia, thrombocytopenia, & neutropenia? Aplastic Anemia
What is the anemic classification (morphology) of Aplastic Anemia? Normocytic Normochromic
What is the term for depression of all cell lines? Pancytopenia
Marrow depression of RBC Red Cell Aplasia
Marrow depression of Granulocytes Agranulocytosis
Marrow depression of Megakaryocytes (platelet suppression) Thrombocytopenia
What Diminished Erythropoiesis is characterized by marrow failure secondary to extensive replacement of marrow by TUMOR or other Lesions? Myelophthisic Anemia
Name 4 origination of metastatic cancer leading to Myelophthisic Anemia Breast, Lung, Prostate, & Thyroid
What is the term for increased concentration of Red Blood cells? Polycythemia (Erythrocytosis)
Term for decreased plasma volume leading to higher RBCs. Relative Polycythemia
Increase in RBCs without decrease in plasma volume Absolute Polycythemia
Absolute Polycythemia may result from primary Neoplastic proliferation of myeloid cells, what is this called? Polycythemia Vera
Name the 2 Disorders associated with White Blood Cells (WBC) NonNeoplastic Disorder & Neoplastic Disorder
Name 3 types of NonNeoplastic Disorders 1. Leukopenia 2. Reactive Leukocytosis 3. Reactive Lymphadenitis
Decrease in number of circulating WBC Leukopenia
Decrease in number of circulating Neutrophils, typically associated with Leukopenia Neutropenia
Severe Neutropenia can result in reduction of granulocytes Agranulocytosis (aka Agranulocytic Angina)
Manifestation by sudden rise of fever, dysphagia, chills, and malaise are characteristics of what? Agranulocytic Angina
What is the treatment of Agranulocytosis? Administration of Granulocyte Colony-Stimulating Factor (G-CSF)
Term for NonNeoplastic Disorder of increased WBC that occurs due to Inflammation as a result of infection. Reactive Leukocytosis
What is the term for a Reactive Leukocytosis that is due to a virus, typically transferred through contact with a nick name of "Kissing Disease"? What's the name of that virus? Infectious Mononucleosis. Epstein-Barr Virus (EBV)
Term for Proliferation of atypical lymphocytes that flood the blood due to Infectious Mononucleosis. Lymphadenopathy
Name the clinical characteristics of Lymphadenopathy Enlarged tonsils, oropharyngeal lymph tissue, spleen, liver, and brain cells.
What is the diagnostic Test for Infectious Mononucleosis that demonstrate Antibodies against EBV? Monospot
Term for NonNeoplastic Disorder characterized by INFECTION and NONmicrobial inflammatory stimuli that causes increased lymphocytes in the blood (lymphocytosis) and enlarged Lymph nodes. Reactive Lymphadenitis
3 patterns of chronic Reactive Lymphadenitis 1. Follicular hyperplasia 2. Paracortical hyperplasia 3. Sinus Histiocytosis
This term implies the activation of B-lymphocytes (autoimmune caused like Rheumatoid Arthritis, Toxoplasmosis, HIV infection) Follicular hyperplasia
This term implies the activation of T-lymphocytes (triggered by antigen like viruses or drug induced) Paracortical hyperplasia
This term implies Hypertrophy of Sinus lining endothelial cells and infiltration of histiocytes (lymph node draining cancer & immune response to tumor) Sinus Histiocytosis
Important infection that results in Reactive Lymphadenitis, 90% younger than 18 yrs, initiated by a cat or splinter/thorn puncture causes this disease. What is the name of the bacteria that causes this? Cat Scratch Disease. Bartonella Henselae
Clinical manifestation of Cat Scratch Disease Inflammatory skin lesion and lymph node enlargement
Neoplastic Disorders are divided into 3 broad categories 1. Lymphoid Neoplasm 2. Myeloid Neoplasm 3. Histiocytic Neoplasm
4 main Lymphoid Neoplasm Hodgkin Lymphoma, NonHodgkin Lymphoma, Lyphocytic Leukemia, Plasma Cell Dyscrasias
3 types of Lymphoid Neoplasm based on Origin (tissue) Lymphoma (Hodgkin & non-Hodgkin), Leukemia & Plasma Cells Dyscrasias
Neoplasm that typically originate from Lymph nodes or other Organs Lymphoma (Hodgkin & non-Hodgkin)
Neoplasm that originate from Bone that spread to circulating blood Leukemia
Neoplasm that originate form Immunoglobulin-secreting Plasma Cells, this can also arise from bone. Plasma Cell Cyscrasias
Term for Lymphoma that form tumor Nodules (originates in germinal centers, found in 40% of non-Hodgkin lymphoma) Follicular Lymphoma
Term for Lymphoma that proliferates in Sheets Diffuse Lymphoma
Immature lymphocytes, typically B or T cells Lymphoblasts (lymphoblastic)
Referring to AGGRESSIVE TUMOR of IMMATURE B or T - cells that occurs predominantly in children and young adults. Lymphoblastic Lymphoma & Lymphoblastic Leukemia (Lymphoblastic Leukemia/Lymphoma)
Lymphoma typically arising from marrow that's is aggressive in nature. Acute Lymphoblastic Leukemia [ALL] ( Acute = rapid/aggressive, Lymphoblastic = B/T cells in nature, Leukemia = marrow of origin)
This resembles ALL but arises from Myeloblast, not B or T cells, from bone marrow. Acute Myeloblastic Leukemia [AML] ( Acute = rapid/aggressive, Myeloblastic = myeloid cells, Leukemia = marrow in origin)
ALL and AML, no matter the origin will produce what type of cell? What do they do? Produce Immature Cells. They FILL the marrow and SUPPRESS hematopoietic stem cells.
Clinical Characteristics of Acute Leukemia (ALL & AML) (5) 1. Abrupt onset of symptoms 2. Symptoms Related to Marrow Suppression 3. Pain/tenderness in Bone 4. ENLARGEMENT (lymph, spleen, liver) 5. CNS symptoms
Typical WBC count in patients with ALL & AML. High WBC count (>100K)
What specific and important cell type are in ALL & AML? What is the term when these are found in peripheral blood and flooded in marrow? Blasts (immature cells). Aleukemic Leukemia.
Which Acute Leukemia has the best prognosis for children 2-10 yrs old (80% curable)? Acute Lymphoblastic Leukemia (ALL) [think: lymphoBlasTic = B for B cell, T for T cell)
What is the term for Neoplastic Mature B-cells? Lymphocytic [think: lymphoCYTIC = CYTIC for Mature B-cell]
Terms for Neoplastic Mature B-cells that originate in Bone and Lymph (2) Chronic Lymphocytic Leukemia (SLL) & Small Lymphocytic Lymphoma (CLL)
SLL & CLL only differ what peripheral blood involvement? SLL - without circulating Mature B-cell. CLL - large number of circulating Mature B-cell
Term for patients who develop antibodies for native RBC Autoimmune Hemolytic Anemia (note: this should be included in the hemolytic anemia category but was only mentioned once in the WBC category)
This type of Lymphoma represents a group of Large B-cell tumors that present with diffuse growth pattern and aggressiveness, found in 50% of non-Hodgkin lymphomas. Diffuse Large B-cell Lymphoma
This lymphoma is a unique B-cell Neoplasm that demonstrate distinct histopathologic (specific tissue characteristic) and clinical features linked specifically to EBV. Burkitt Lymphoma
The Histopathology of Burkitt Lymphoma demonstrates a High MITOTIC rate and SHEETS of lymphocytes mixed with benign Histiocytes, what is this term? Starry Sky
Burkitt Lymphoma is commonly referred to as ____ due to neoplasm presenting as large Osteolytic lesion in the maxilla or mandible. (common in Africa) African Jaw Disease
Group of B-cell neoplasm that form a single clone of immunoglobulin-secreting cells resulting in increase of single immunoglobulin in the serum. (15% death, typically middle age-elderly) Plasma Cell Dyscrasias
2 types of Plasma Cell Dyscrasias Multiple Myeloma & Plasmacytoma
Most common type of Plasma Cell Dyscrasias, malignant proliferation of a Single Clone of Plasma Cell. Multiple Myeloma
Multiple Myeloma excrete what two types of proteins? Bence Jones Protein & Monoclonal Gammopathy
This Multiple Myeloma protein excretion is characterized by excess light chains. Bence Jones Protein
This Multiple Myeloma protein excretion typically consist of IgG. Monoclonal Gammopathy
What is the median age for diagnosing Multiple Myeloma? 70 yrs old.
5 Major clinical presentation of Multiple Myeloma 1. Bone pain & hypercalcemia 2. Recurrent Bacterial Infection 3. Renal Insufficiency 4. Amyloidosis (inappropriately folded proteins) 5. Hyperviscosity Syndrome (excess protein in blood)
This Plasma Cell Dyscrasias refers to a single monoclonal plasma cells proliferating in the skeleton or soft tissue. Plasmacytoma
What cells distinguishes Hodgkin Lymphoma from other lymphomas? Reed-Sternberg Cells (R-S cells)
Term for Altered R-S cells demonstrating abundant, pale-staining Cytoplasm Lacunar Cells
Term for Altered R-S cells that exhibit Multilobed, Puffy nuclei. Popcorn Cells
Clinical characteristics of Hodgkin Lymphoma Weight loss, weakness, fever, night sweats, pruritus (itching) and anemia.
2 tumors that involve peripheral CD4+ T-cells of the SKIN Mycosis Fungoides & Sezary Syndrome
Skin lesions begins as plaques that enlarge into nodules and with progression often spreading to lymph nodes and viscera. Mycosis Fungoides
Generalized Exfoliative Erythroderma with Leukemic involvement, survival rate of 1-3 yrs. Sezary Syndrome
This is a Neoplastic Disorder that arises from Hematopoietic stem cells that proliferate and replace normal bone marrow. Myeloid Neoplasm
3 major categories of Myeloid Neoplasm 1. Acute Myeloid Leukemia (AML) 2. Chronic Myeloproliferative Disorders 3. Myelodyplastic Syndrome (not discussed)
AML is a malignancy of immature myeloid cells (myeloblasts), with a Characteristic Tumor-like mass Granulocytic Sarcoma (Chloroma)
With AML alone, "good risk" patients has a __% long term disease free survival. AML with other risks involved has a __% long term disease free survival. 50% & 15-30%
Disorder that arises from Multipotent Myeloid stem cells Chronic Myeloproliferative Disorder
4 types of Myeloproliferative Disorder 1. Chronic Myeloid Leukemia 2. Polycythemia Vera 3. Primary Myelofibrosis 4. Essential Thrombocytopenia
This type of Myeloproliferative disorder is thought to be neoplasm of Megakarycytes that releases Fibroblastic Growth factor resulting in diffuse fibrosis in marrow spaces. Primary Myelofibrosis
Leukemia of marrow stem cells in which Granulocytic precursors predominate (neutrophils, eosinophils, and/or basophils) Chronic Myeloid Leukemia
Infection of Healthy Patients may trigger a similar pattern of Chronic Myeloid Leukemia but this is not cancer-related mutated genes. Leukemoid Reaction
The accelerated phase of Leukemia will transform WBC to immature cells and become less responsive to therapy. This presentation resemble AML and is termed what? Blast (immature cells) Crisis
Term for Leukemia of marrow stem cells in which Erythroid Precursors Doinate (Characteristic: Myeloid cells, Absolute Polycythemia, increase in RBC production, Neoplastic) Polycythemia Vera (refer to Polycythemia Section)
Term for Histiocytic Neoplasm are often mixed with Eosinophils, nicknamed Eosinophilic Granuloma, often presenting with 3 distinct pattern. Langerhans Cell Histiocytocis (Histiocytosis X)
Langerhan Cells under electron microscopy (EM) is identified by a tennis racket-like cytoplasmic inclusion. Birbeck Granule
3 distinct pattern of Langerhans Cell Histiocytocis (Hystiocytosis X) 1. Acute Multisystem Langerhans Cell Histiocytosis (Letterer-Siwe Disease) 2. Chronic Multifocal Langerhand Cell Histiocytosis (Hand-Schuller-Christian Disease) 3. Unifocal Langerhands Histiocytosis (Eosinophilic Granuloma)
This Langerhan cell histiocytosis typically occurs before 2 yrs of age, widespread skin lesion, hepatosplenomegaly, lymphadenopathy, lung lesions, and osteolytic bone lesions (50% survival after 5 yrs from chemotherapy) Latterer-Siwe Disease
This Langerhan cells histiocytosis usually affects children involving skull, ribs, and femur, 50% involve pituitary gland leading to diabetes and Exophthalmos (eye bulging). Hand-Schuller-Christian Disease
The combination of skull, lesions, diabetes & Exophthalmos is termed... Hand-Schuller-Christian Triad
This Langerhan cells histiocytosis presents as a unifocal bone lesion, may or may not be symptomatic or cause pain and tenderness. Eosinophilic Granuloma
Name the 4 types of Bleeding Disorders 1. Disseminated Intravascular Coagulation 2. Thrombocytopenia 3. Immune Thrombocytopenia Purpura (ITP) 4. Heparin-induced Thrombocytopenia
Name the 5 Diagnostic tests for Bleeding Disorders 1. Bleeding Time 2. Platelet Count 3. Coagulation Time 4. Prothrombin Time (PT) 5. Partial Thromboplastin Time (PTT)
Represents the time for standardized skin puncture to stop bleeding, this is a Clinical test of platelet function. Bleeding Time
A Laboratory Test to detect quantitative deficiency in platelets Platelet Count
Measurement of the time it takes for whole blood to clot when placed in a glass test tube, Clinical Test of intrinsic system. Coagulation time
A Laboratory Test that measure the time for blood to clot in the presence of Thromboplastin, functions of extrinsic and common coagulation pathway. Prothrombin time (PT)
A Laboratory Test that measures the time for blood to clot upon activation of factor XII, tests the function of intrinsic and common coagulation pathway. Partial Thromboplastin Time (PTT)
This Bleeding Disorder is characterized by activation of coagulation system with formation of thrombi throughout the microcirculation. Disseminated Intravascular Coagulation (DIC)
Term for widespread thrombosis with secondary consumption of platelets and coagulation factors and activation of fibrinolytic system. Consumption Coagulopathy
This Bleeding Disorder exhibits a Decreased Platelet, leading to prolong bleeding time with normal coagulation time, PT and PTT (platelet count below 150K), spontaneous bleeding may occur below 20K. Thrombocytopenia
This Bleeding Disorder is also called Idiopathic Thrombocytopenia Purpura, arising from development of Antiplatelet Immunoglobulins secondary to SLE or Lymphoma. Immune Thrombocytopenic Purpura (ITP)
This Bleeding Disorder is due to prolonged use of unfractionated heparin leading to development of antiplatelet antibodies, leads to widespread thrombosis. Heparin-induced Thrombocytopenia
Name the 3 types of Coagulation Disorders 1. von Willebrand Disease 2. Factor VIII Deficiency 3. Factor IX Deficiency
Autosomal Dominant disorder characterized by reduced levels of von Willebrand Factor, also used to stabilize Factor VIII. This disorder results in increased coagulation time. von Willebrand Disease
X-linked recessive disorder that results in reduced amounts or activity of Factor VIII, most common hereditary Bleeding Disorder. Factor VIII Deficiency
What is another name for Factor VIII Deficiency? Classic Hemophilia - Hemophilia A
This Bleeding Disorder is similar to Classic Hemophilia excep the deficiency is Factor IX rather than Factor VIII. Factor IX Deficiency
What is another name for Factor IX Deficiency? Christmas Disease - Hemophilia B
Created by: ddde227