Things I've Wikied

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Wilms Tumor

Back 1

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.

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WAGR Syndrome

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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

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Burkitt's Lymphoma

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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.

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Ewing's Sarcoma

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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.

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CD20

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expressed on the surface of all mature B-cells.

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BCR-ABL Mutation

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Chronic myelogenous leukemia

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Philadelphia chromosome

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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.

Front 8

Cromolyn sodium

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Stabilize mast cells - e.g. prevent histamine degranulation in asthma.

Front 9

Drug interaction with 6-mercaptopurine?

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Allopurinol - decreases clearence of 6-MP and increases its toxicity

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Rheumatic fever pathogenesis

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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.

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Histiocytosis

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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.

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Hand-Foot and Mouth

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Coxsackie virus

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Erythema multiforme

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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.

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Stevens-Johnson Stndrome

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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

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Anisocytosis

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Red blood cells are of unequal SIZE. This is found in anemia and other blood conditions.

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poikilocytosis

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RBCs of unequal SHAPE

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hypersegmented nuclei in PMN

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B12 or folate deficiency

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Pellagra

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Vitamin B3 Deficiency

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4 D's of Pellagra

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Diarrhea, Dimentia, Dermatitis, Doom!

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Vitamin B3 (name)

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Niacin

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Acanthocytes

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Liver Disease

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Spindle like projections

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Anisocytosis

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IDA

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Variable RBC sizes (increased RDW)

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Basophilic stippling

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Thalassemia, lead poisoning

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Dark purple granules in cytoplasm (aggr, ribosomes)

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Darcocytes

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Myelofibrosis, bone marrow infiltration

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teardrop-shaped cells

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Peripheral Smear:
Dimorphism

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MDS

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Microcytes and macrocytes

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Elliptocytes

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IDA, hereditary elliptocytosis

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spectrin mutations

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Heinz bodies

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Post splenectomy, G6PD

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Inclusion bodies of denatured Hb

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Howell-Jolly bodies

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After splenectomy

Hint 28

remnants of red cell nucleus

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Hypochromia

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IDA

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Macrocytosis

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Liver dz, ETOH, hypothyroid, medications, B12 or Folate def.

Hint 30

increased MCV

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Microcytosis

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IDA, thalassemia, sideroblastic anemia

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Oval macrocytes

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B12 and folate def. medications causing megaloblastic anemias

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Oval large RBCs

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Pappenheimer bodies

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After splenectomy

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mixed precipitates of iron, mitochondria, ribosomes

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Pencil cells (cigar cells)

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IDA

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Poikilocytosis

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IDA

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variable appearence

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Polychromasia

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Hemolysis or acute bleed

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circulating reticulocytes

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Round macrocytes

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Liver dz, ETOH, hypothyroidism causing non-Megaloblastic Anemias

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Round large RBCs

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Sickle cells

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Sickel cell Disease

Hint 38

glutamic acid to valine mutation in B-chain Hb

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Schistocytes

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Valvular heart disease, DIC, HUS, TTP

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fragmented red blood cells

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Spherocytes

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Autoimmune hemolysis, hereditary spherocytosis

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Target-cells

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Liver Dz, IDA, thalassemias

Front 42

Alloimmunization

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lloimmunity is a condition in which the body gains immunity, from another individual of the same species, against its own cells.

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RANTES

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Regulated upon Activation, Normal T-cell Expressed, and Secreted

producedd platelets and causing allergic transfusion rxn

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Fanconi Anemia

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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.

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TTP

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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

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Bernard-Soulier Syndrome

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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

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SLE Criteria
(4 of 11 for dx)
DAMP AS RHINO

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Discoid rash
Arthritis
Malar rash
Photosensitivity

ANA positive
Serositis

Renal disorder
Hematologic disorder
Immunologic disorder
Neurologic disorder
Oral ulcers

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Bernard-Soulier Dz

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Deficiency of GP Ib

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Von Willibrand Dz

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Deficiency of vWF

vWF binds to collagen and binds GP Ib on platelet.

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Glanzmann's Thrombasthenia

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Deficiency of GP IIb/IIIa