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50 Cards in this Set

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Wilms Tumor
Wilms' tumor is a neoplasm of the kidneys that typically occurs in children. It is eponymously named after Dr. Max Wilms [1], a German surgeon (1867-1918) who first identified this form of cancer. It is also known as a nephroblastoma.
WAGR Syndrome
Genetic syndrome
Affected children are predisposed to develop:
Wilms tumor (a tumor of the kidneys)
Aniridia (absence of the colored part of the eye, the iris)
Genitourinary anomalies (gonadoblastoma)
mental Retardation
Burkitt's Lymphoma
Burkitt's lymphoma (or "Burkitt's tumor", or "Malignant lymphoma, Burkitt's type") is a cancer of the lymphatic system (in particular, B lymphocytes). It is associated with the Epstein-Barr virus, also the cause of mononucleosis as well as other cancers. It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1956 while working in equatorial Africa.

Children affected with the disease often also had chronic malaria which is believed to have reduced resistance to the virus. This is known as classical African or endemic Burkitt's lymphoma.
Ewing's Sarcoma
Ewing's sarcoma is the common name for primitive neuroectodermal tumor. It is a rare disease in which cancer cells are found in the bone or in soft tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, and the ribs. James Ewing (1866-1943) first described the tumor, establishing that the disease was separate from lymphoma and other types of cancer known at that time. Ewing's sarcoma occurs most frequently in male teenagers. Ewing's sarcoma is the result of a translocation between chromosomes 11 and 22, which fuses the EWS gene of chromosome 22 to the FLI1 gene of chromosome 11.
CD20
expressed on the surface of all mature B-cells.
BCR-ABL Mutation
Chronic myelogenous leukemia
Philadelphia chromosome
The exact chromosomal defect in Philadelphia chromosome is translocation. Parts of two chromosomes, 9 and 22, swap places. The result is that part of the BCR ("breakpoint cluster region") gene from chromosome 22 (region q11) is fused with part of the ABL gene on chromosome 9 (region q34). Abl stands for "Abelson", the name of a leukemia virus which carries a similar protein.

The result of the translocation is a protein of p210 or sometimes p185 weight (p is a weight fraction of cellular proteins in kDa). The fused "bcr-abl" gene is located on the resulting, shorter chromosome 22. Because abl carries a domain that can add phosphate groups to tyrosine residues (tyrosine kinase) the bcr-abl fusion gene is also a tyrosine kinase.
Cromolyn sodium
Stabilize mast cells - e.g. prevent histamine degranulation in asthma.
Drug interaction with 6-mercaptopurine?
Allopurinol - decreases clearence of 6-MP and increases its toxicity
Rheumatic fever pathogenesis
t is strongly suspected that acute rheumatic fever is a hypersensitivity reaction induced by group A streptococci, but the exact pathogenesis remains uncertain despite many years of investigation.[83] It is thought that antibodies directed against the M proteins of certain strains of streptococci cross-react with glycoprotein antigens in the heart, joints, and other tissues. The onset of symptoms 2 to 3 weeks after infection and the absence of streptococci from the lesions support the concept that RF results from an immune response against the offending bacteria.
Histiocytosis
Though histiocytosis can refer to any of several specific diseases, the term is generally used to refer to a rare blood disease that is caused by an excess of white blood cells called histiocytes.
Pulmonary Histiocytosis X, Histiocytosis X, Eosinophilic Granuloma, Nonlipid Reticuloendotheliosis, Langerhans cell histiocytosis, Letterer-Siwe Disease, Hand-Schuller-Christian Disease, Pulmonary Langerhans Granulomatosis, LCH.
Hand-Foot and Mouth
Coxsackie virus
Erythema multiforme
Erythema multiforme is a skin condition of unknown etiology that usually follows an antecedent infection or drug exposure. It varies from a mild, self-limited rash (E. multiforme minor) to a severe, life-threatening form (E. multiforme major, or Stevens-Johnson syndrome) that also involves mucous membranes.
Stevens-Johnson Stndrome
Stevens-Johnson syndrome (SJS) is a severe and life-threatening condition. It is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. SJS has been classified as a severe expression of erythema multiforme (EM), and is sometimes referred to as erythema multiforme major.

Cases involving greater than 30% of body surface area are termed Toxic Epidermal Necrolysis Syndrome
Anisocytosis
Red blood cells are of unequal SIZE. This is found in anemia and other blood conditions.
poikilocytosis
RBCs of unequal SHAPE
hypersegmented nuclei in PMN
B12 or folate deficiency
Pellagra
Vitamin B3 Deficiency
4 D's of Pellagra
Diarrhea, Dimentia, Dermatitis, Doom!
Vitamin B3 (name)
Niacin
Acanthocytes
Liver Disease
Spindle like projections
Anisocytosis
IDA
Variable RBC sizes (increased RDW)
Basophilic stippling
Thalassemia, lead poisoning
Dark purple granules in cytoplasm (aggr, ribosomes)
Darcocytes
Myelofibrosis, bone marrow infiltration
teardrop-shaped cells
Peripheral Smear:
Dimorphism
MDS
Microcytes and macrocytes
Elliptocytes
IDA, hereditary elliptocytosis
spectrin mutations
Heinz bodies
Post splenectomy, G6PD
Inclusion bodies of denatured Hb
Howell-Jolly bodies
After splenectomy
remnants of red cell nucleus
Hypochromia
IDA
Macrocytosis
Liver dz, ETOH, hypothyroid, medications, B12 or Folate def.
increased MCV
Microcytosis
IDA, thalassemia, sideroblastic anemia
Oval macrocytes
B12 and folate def. medications causing megaloblastic anemias
Oval large RBCs
Pappenheimer bodies
After splenectomy
mixed precipitates of iron, mitochondria, ribosomes
Pencil cells (cigar cells)
IDA
Poikilocytosis
IDA
variable appearence
Polychromasia
Hemolysis or acute bleed
circulating reticulocytes
Round macrocytes
Liver dz, ETOH, hypothyroidism causing non-Megaloblastic Anemias
Round large RBCs
Sickle cells
Sickel cell Disease
glutamic acid to valine mutation in B-chain Hb
Schistocytes
Valvular heart disease, DIC, HUS, TTP
fragmented red blood cells
Spherocytes
Autoimmune hemolysis, hereditary spherocytosis
Target-cells
Liver Dz, IDA, thalassemias
Alloimmunization
lloimmunity is a condition in which the body gains immunity, from another individual of the same species, against its own cells.
RANTES
Regulated upon Activation, Normal T-cell Expressed, and Secreted

producedd platelets and causing allergic transfusion rxn
Fanconi Anemia
Genetic disease affecting children and adults. Characterized by short stature, skeletal anomalies, increased incidence of solid tumors and leukemias, bone marrow failure (aplastic anemia), and cellular sensitivity to DNA damaging agents such as mitomycin C.
TTP
Thrombotic thrombocytopenic purpura
Most cases of TTP arise from deficiency or inhibition of the enzyme ADAMTS13, which is responsible for cleaving large multimers of von Willebrand factor. This leads to hemolysis and end-organ damage, and may require plasmapheresis therapy
Bernard-Soulier Syndrome
AR
Absent or decreased expression of glycoprotein Ib/IX/V complex on platelets. This complex is the receptor for von Willebrand factor (vWF), and deficient binding of vWF to the platelet membrane at sites of vascular injury, results in defective platelet adhesion
SLE Criteria
(4 of 11 for dx)
DAMP AS RHINO
Discoid rash
Arthritis
Malar rash
Photosensitivity

ANA positive
Serositis

Renal disorder
Hematologic disorder
Immunologic disorder
Neurologic disorder
Oral ulcers
Bernard-Soulier Dz
Deficiency of GP Ib
Von Willibrand Dz
Deficiency of vWF

vWF binds to collagen and binds GP Ib on platelet.
Glanzmann's Thrombasthenia
Deficiency of GP IIb/IIIa