Blood Disorders - Final Exam Flashcards
Fluid of blood
Plasma
Cells of blood
WBC (leukocytes)
RBC (erythrocytes)
Platelets (thrombocytes)
Sites of hematopoiesis
Adults: calvarium, vertebrae, ribs, sternum, pelvis
Children: most bones
Fetus: most ones
Embryo: yolk sac, liver cords
Centrifuging of blood
Plasma at top
Buffy coat (WBC and platelets) in middle
RBC at bottom
Erythrocytes
For transportation of oxygen from lungs into peripheral tissues
Live in circulation for 120 days - broken down in spleen
Hemoglobin
Complex molecules consisting of four heme groups (Fe in middle) and four globins
97% Hemoglobin A
Fetal hemoglobin
Alpha chains and gamma chains
In children, traces in adults
Bilirubin
Heme is broken down and Fe is removed
Hemoglobin synthesis
Requires iron, Vit B12, Vit B6, folic acid
Erythropoietin
Kidney sensing O2 is low and secrete hormone to tell bone marrow to make more RBC
Thrombocytes
Platelets
Essential clotting factors
Malignant transformation of hematopoietic cells (2)
Solid tumors or leukemia
Anemia (3)
Reduction of Hb in blood - below normal levels
Less than reference range established in given population
Associated with appearance of abnormal Hb, reduction in RBC, structural abnormalities of RBC
Causes of anemia (3)
- Decreased hematopoiesis
- Abnormal hematopoiesis
- Increased loss or destruction of RBC
Decreased hematopoiesis (2)
- Bone marrow failure (aplastic anemia, myelophthisic anemia)
- Deficiencies of nutrients (vit B12, folic acid, protein)
Abnormal hematopoiesis
Usually consequence of genetic abnormalities
Sickle cell anemia
Increased loss and destruction of RBC (4)
- Bleeding
- Intrasplenic sequestration
- Immune hemolysis
- Infections
Aplastic anemia
symptoms: 5
Idiopathic, secondary
Bone marrow depletion of hematopoietic cells (consists only of fibroblasts, fat cells, scattered lymphocytes)
Anemia, leukopenia, thrombocytopenis
Uncontrollable infections, bleeding tendency, chronic fatigue, sleepiness, weakness
Iron deficiency anemia
(histology: 2
causes: 3)
Most common form
Hypochromic (pale), microcytic (small) RBC
Increased loss of iron, inadequate iron intake or absorption, increased iron requirements
Megablastic anemia
histology: 3
Caused by deficiency of vit B12 or folic acid
Large RBC (macrocytic anemia)
Bone marrow is hypercellular with numerous megaloblasts (abnormal RBC precursors)
Hypersegmentation of neutrophils
Vit B12 deficiency (3)
- Pernicious anemia
- Lack of gastric intrinsic factor
- Atrophic gastritis
Folic acid deficiency
Inadequate intake
Due to diet or malabsorption
Hemolytic anemia (4)
- Increased RBC destruction
- Intracorpuscular or extracorpuscular defects
- Compensatory erythroid hyperplasia of BM
- Hyperbilirubinemia (jaundice)
Intracorpuscular defects (3)
- Structural abnormalities
- Sickle cell anemia, thalassemia, hereditary spherocytosis
- Infection
Extracorpuscular defects
- Antibodies, infectious agents, mechanical factors
- Autoimmune hemolytic anemia, hemolytic disease of newborn, transfusion reactions, hemolytic anemia due to cardiac valve prosthesis, disseminated intravascular coagulation
Sickle cell anemia
Substitution of glutamic acid by valine Synthesis of abnormal beta chain of globin Most prevalent among African populations Multiple infarctions in various organs Jaundice
Sickle cell anemia - low oxygen
Hemoglobin S polymerizes and forms chain that deforms cells
Reversible if O2 is back up, quickly
All symptoms occur due to clotting
Long term complications of sickle cell anemia (3)
- Delayed intellectual development, neurologic deficits
Largely avoided due to medical surveillance and appropriate management or crises - Cardiopulmonary insufficiency
- Recurrent infections
Thalassemia
(histology: 1
symptoms: 5
developmental: 2)
Genetic defect in synthesis of HbA, do not produce enough
Reduced rate of globin chain synthesis
Beta or alpha chain affected
Minor (heterozygotes) or major (homozygotes, very serious)
Hypochromic (pale RBC) anemia
Splengomegali, hemosiderosis, hepatomegaly
Bone marrow: compensatory hyperplasia
Calvarium (crew cut) hair on radiographic study - a lot of hematopoiesis in skull and changes the way it looks
Jaundice
Chronic anemia that slows growth of children
Impaired normal intellectual development
Cardiorespiratory insufficiency
Immune hemolytic anemias
Mediated by antibodies that destroy RBC (autoantigens, alloantigens, neoantigens)
Mismatched blood transfusion
Hemolytic disease of new bown
Autoimmune hemolytic anemias
Neoantigens
Caused by penicillin getting absorbed into RBC
Polycythemia vera
Too much Hb
Primary + secondary
Erythrocytosis
Increased number of RBC
Primary Polycythemia
Cancer
Myeloproliferative disorder
Clonal proliferation of hematopoietic stem cells
Uncontrolled production of RBC
Secondary Polycythemia
Increased RBC volume due to erythroid bone marrow hyperplasia caused by erythropoietin
Usually caused by prolonged hypoxia
Risk factors of secondary polycythemia (4)
- Living at high altitudes
- Anoxia secondary to chronic lung disease
- Congenital heart disease
- Renal carcinoma
Polycythemia symptoms (7)
- Hypertension
- Dark red or flushed face
- Headaches
- Visual problems
- Neurologic symptoms
- Splegomegaly
- Hypercellular bone marrow
Leukocytic disorders (4)
- Leukopenia
- Leukocytosis
- Leukemia
- Lymphoma
Leukopenia
Reduction in WBC
Neutropenia or lymphopenia
Neutropenia
Reduction in neutrophils
Caused by bacterial infections or drugs
Lymphopenia
Reduction in lymphocytes
Bacteria, viral, fungal, parasitic infections
Leukocytosis
Increased number of WBC in peripheral blood
Neutrophilia, eosinophilia, lymphocytosis, splengomegaly, lymphadenopathy
Neutrophilia
Increased WBC
due to bacterial infection
Eosinophilia
Increased WBC due to allergies, some skin diseases, parasitic infections
Lymphocytosis
Increased WBC due to viral infections, chronic infections, some autoimmune disorders
Malignant disease of WBC (3)
- Leukemias
- Lymphomas
- Plasma cell myeloma
Leukemia
symptoms: 3
Malignant disease involving WBC or their precursors in BM, peripheral blood
Anemia, recurrent infections, uncontrollable bleeding
Myeloid or lymphoid
Myeloid
From neutrophil, basophil, etc
Lymphoid
B or T cells
Lymphomas
locations: 5
Lymphoid cell malignant diseases predominantly involving lymph nodes
Most common in adults, aggressive forms in kids
Malignant cells infiltrate lymph nodes, spleen, thymus or BM or extranodal spread
Non-Hodgkin’s lymphoma
B cell, T cell, NK cell
Most have B cell phenotype
All age groups (more common in adults)
Can spill over in blood and present as leukemia
Hodgkin lymphoma
B cell only Bimodal age distribution (25 +55) 5 types Reed-Sternberg cells present Enlarged lymph nodes Stage I and II have excellent prognosis Extranodal involvement and leukemic spread is rare
Plasma cell myeloma
Malignant disease of plasma cells
High level of antibodies
Most patients older than 45
Malignant cells proliferate in BM
HTLV-1
Causes lymphoma cancer
EBV
Causes Burkitt’s lymphoma
t(8,14)***
Burkitt’s lymphoma
t(9,22)***
Chronic myelogenous leukemia
Philadelphia chromosome
t(15,17)
Acute promyelocytic leukemia
Acute lymphoblastic leukemia
treatment: 1
Immature form of cells
Very serious, can die immediately without treatment
Most common in children, almost always B cell origin
Massive infiltration of BM and peripheral blood with immature lymphoid cells (B cell blasts)
Modern chemotherapy
Chronic myelogenous leukemia
treatment: 1
Mature type of cell
BM, peripheral blood overrunn with neutrophils and precursors
Three phases (chronic phase, accelerated phase, blast crisis)
Treatment with tyrosine kinase inhibitors
Philadelphia chromosome with BCR-ABL gene rearrangement (t9,22)
Age distribution of leukemias
Rate goes up with age
except acute lymphocytic
Acute myelogenous leukemia
Most common form of acute leukemia in adults
Clonal proliferation of myeloid precursors in BM (abnormal cells or cytogenic abnormalities)
Without treatment, most patients due within 6 months after symptom onset
Chronic lymphocytic leukeumia
Malignant disease involving lymphocytes, mainly B cells
Most patients older than 50
Should be expected if lymphocytes are >5000/mL
Medial survival: 7-9 years after diagnosis
Follicular lymphoma
symptoms: 2
Most common form of lymphoma in USA (45%)
Mostly seen in older people
Slow growing
Presents with long-standing elargement of lymphnodes, mild constitutional symptoms
Median survival: 7-9 years
Diffuse large-cell lymphomas
Most aggressive form of NHL
Tissue infiltration by large lymphoid cells with irregular nuclear outlines, prominent nucleoli
Complete remission in 75% of patients with chemotherapy
Burkitt’s Lymphoma
Highly malignant tumour composed of small B cells
Extranodal masses
Endemic or sporadic variants
Most children and young adults can be cured
Endemic variant of Burkitt’s lymphoma
Sub-saharan Africa
Children infected with EBV
Mandible, facial soft tissue involvement
Sporadic variant of Burkitt’s lymphoma
Children and young adults
Abdominal masses
5 types of Hodgkin’s lymphoma
- Nodular sclerosing
- Lymphocyte predominant
- Lymphocyte rich
- Mixed cellularity
- Lymphocyte depleted
Reed-Sternberg cells (2)
Bilobed or multilobed nucleus, prominent nucleoli surrounded by clear halo
Multiple myeloma
Plasma cell myeloma
Diagnosis of plasma cell myeloma (4)
- X ray studies
- Serum electrophoresis
- Bone marrow biopsy
- Clinical presentation
Survival rate is less than 3-4 years from diagnosis
Most die from kidney failure and infection
Diagnosis of Non-Hodgkin’s lymphoma (2)
- Light microscopy
2. Ancillary techniques (flow cytometry, immunositochemistry, cytogenetic analysis)
Clinical features of Non-Hodgkin’s lymphoma (3)
- Lymph node enlargement
- Systemic consitutional symptoms
- Extranodal tumor spread
Symptoms of plasma cell myeloma (5)
- Lytic lesions (punched out holes) in calvaria vertebrae
- Hypercalcemia (osteoclast function)
- Renal failure
- Anemia
- Leukopenia