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48 Cards in this Set
- Front
- Back
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Dystrophin
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the protein that mutated in Becker's and Duchenne's muscular dystrophy.
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Hereditary spherocytosis
defect protein, lab, treatment |
ankyrin, or spectrin defect. RBC skeleton and membrane problem. Round and small RBC no central pallor.
MCHC increase, red cell distribution width increase. RBC premature removal by spleen. family history splenomegaly, aplastic crisis (Parovirus B19 infection) lab:positive osmotic fragility test. normal to low MCV with abundance of cells which may mask microcytia treatment: splenectomy, need pneumococcal vaccine before surgery. may need folic acid supplement |
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Follicular lymphoma
Adults or kid? translocation, and what genes |
adult
t(14,18) Heavy chain Ig and bcl-2 (inhibit apoptosis) |
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Mantle cell lymphoma
Adult? translocation, genes CD |
older male
t(11,14), cyclin D1 and Heavy chain Ig poor prognosis CD5+ |
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Diffuse large B-cell lymphoma
Adult? translocation genes? |
usually older adults, 20% in kids,
most common adult NHL maybe mature T cell in origin (20%) |
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Burkitt's lymphoma
adults? translocation, genes pathology |
Adolescents
t(8,14), c-myc (transcription factor) and heavy chain Ig starry sky in LM, sheets of lymphocytes with interspersed macrophage associated with EBV Jaw lesion endemic form in Africa, pelvis or abdomen in sporadic form |
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H pylori
gastric ulcer or duodenal ulcer risk factor for diagnostic test treatment |
70% gastric ulcer, 100% duodenal ulcer
peptic ulcer, gastric adenocarcinoma, lymphoma!! Urease positive, creast alkaline environment. triple therapy: proton pump inhibitor, clarithromycin, amoxicillin or metronidazole |
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acute inflammatory demyelinating polyradiculopathy
Guillain-Barre syndrome symptom, associated infection? lab treatment |
Autoimmune destroy schwann cell, thus inflammation and demyelination of peripheral nerves and motor fibers.
symmetric ascending muscle weakness/paralysis beginning in lower extremities. 面瘫50%, Autonomic function may be affected (cardiac irregularity, hypertension, hypothension) 都survive, recover after weeks or months CSF 蛋白细胞分离， protein increase, papilledema Campylobacter jejuni and CMV treatment: respiratory support, plasmapheresis, IV immunoglobin |
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Methotrexate (MTX)
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Folic acid analog, inhibit dihydrofolate reductase, so DHF can become THF, dUMP can't become dTMP,
Cancer: leukemia, lymphoma, choriocarcinoma, sarcoma non-cancer: abortion, ectopic pregnancy, RA, psoriasis Tox: mylosuppression, reversible with folinic acid (leucovorin), macrovesicular fatty change in liver, mucositis, jaundice (indirect), teratogenic |
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Henoch-Schonlein purpura
classic triad |
Most common childhood systemic vasculitis, Follow URI
triad: skin: palpable purpura on buttocks/legs Arthralgia GI: abdominal pain, melena, multiple lesion of the same age IgA complex deposits, IgA nephropathy |
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IgA nephropathy
Nephritic or nephronic related to what disease LM, EM, IF |
Henoch-Schonlein purpura, childhood vasculitis
LM: mesangial proliferation EM:mesangial IC deposits IF: IgA based IC deposits in mesangial often follow URI or acute gastroenteritis |
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polycythemia vera
RBC, WBC, platelets Phil chromosome, Jak2 |
all linage increase, RBC, WBC platelets,
Phili chromosome negative Jak2 mutation positive independent of EPO |
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Essential thrombocytosis
RBC, WBC, platelets Phil chromosome, Jak2 |
only platelets increase, phil N, Jak2 mutation (30-50%)
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Myelofibrosis
RBC, WBC, platelets Phil chromosome, Jak2 RBC morphology |
RBC decrease, WBC Platelets variable,
phil N, Jak2 mutation (30-50%) teardroop RBC |
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CML
RBC, WBC, platelets Phil chromosome, Jak2 gene transformation |
RBC decrease, WBC platelets increase
phil P, Jak2 mutation N bcr-abl |
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Hereditary thrombosis, hypercoagulability
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Factor V leiden
mutation in prothrombin gene Antithrombin deficiency protein C or S deficiency |
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Factor V leiden
mechanism |
mutated V factor, resistant to degradation by protein C.
most common cause of hypercoagulability in white |
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Prothrombin gene mutation
gene mutation |
mutation in 3' untranslated region, prothrombin production increase, so plasmin level and venous clots increase
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Antithrombin deficiency
mechanism, heparin |
AD, ATIII complex with heparin to break up fibrin and X faster. So when ATIII is not there, heparin doesn't work.
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Protein C or S deficiency
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reduced the ability to inactivate V and VIII,
increase risk of thrombotic skin necrosis with hemorrhage following warfarin. |
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Progressive multifocal leukoencephalopathy PML
virus? mechanism |
demylination of CNS due to destroy of oligodendrocytes.
JC virus. seen in 2-4% AIDS (reaction of latent viral infection). Rapidly progressive, fatal in months |
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Etoposide, carboplatin
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inhibit topoisomerase II,
small cell carcinoma, prostate and testicular carcinoma tox: myelosuppression, GI irritation, alopecia |
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Adult primary brain tumors
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Glioblastoma (grade IV astrocytoma)
Meningioma schwannoma Oligodendroglioma Pituitray adenom |
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Glioblastoma
where, origin, benign or malignant, symptom, LM |
cerebral hemisphere, can cross corpus callosum,
stain astrocytes with GFAP peudopalisading, pleomorphic tumor cells-border central areas of necrosis and hemorrhage |
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Meningioma
where, origin, benign or malignant, symptom, treatment, LM, |
near surface of the brain including the parasagittal region
arise from arachniod cells, external to parenchyma, may have dural attachment benign and resectable LM: spindle cells concentrically arranged in a whorled pattern. psammoma bodies |
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schwannoma
where, origin, benign or malignant, symptom, treatment LM |
schwann cell origin, CN VIII, often at cerebellopotine angle
resectable or treat with sterotactic radiosurgery. S100 + NF2 LM: uniformly spindled Schwann cells with Antoni A (cellular fascicularc成束的c) and Antoni B (myxoid; vacuolated黏液状的,有液泡的) regions |
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Oligodendrogioma
where, origin, benign or malignant, symptom, treatment LM |
rare, slow growing, frontal lobes,
LM: chicken wire capillary, "fried egg", areas of calcification |
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Pituitary adenoma
where, symptom, sequelae |
prolactinoma,
bitemporal hemianopia sequelae: hypo or hyperpituitarism |
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Childhood primary brain tumors
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Pilocytic (low grade) astrocytoma
Medulloblastoma Ependymoma Hemangioblastoma Craniopharyngioma |
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Pilocytic astrocytoma
where, origin, benign or malignant, symptom, treatment LM |
children, often in posterior fossa (e.g. cerebellum). maybe supratentorial. GFAP
Benigh and good prognosis Rosentha fibers cystic+solid |
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Medulloblastoma
where, origin, benign or malignant, symptom, treatment LM |
highly malignant, cerebellum. a form of primitive neuroectodermal tumor. can compress 4th ventricle causing hydrocephalus, can send mets to spinal cord
Homer-Wright rosettes. solid, small blue cells. Radiosensitive |
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Ependymoma
where, origin, benign or malignant, symptom, treatment LM |
4th ventricle, can cause hydrocephalus, poor prognosis
LM: characteristic perivascular pseudorosetts. rod-shaped blepharoplast (basal ciliary bodies) found near nucleus |
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Hemangioblstoma
where, origin, benign or malignant, symptom, treatment LM |
most often cerebellar,
von-Hipple-Lindau syndrom when found with retinal angiomas can produce EPO foamy cells and highly vascularity |
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craniopharyngioma
symptom, location, adult or kid, origin, LM |
present with endocrine dysfunction, most common neoplasma from pituitary region.
derived from Rathke's pouch. calcification |
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sickle cell anemia disease complication
siclle cell trait is heterozygotes (don't go to high altitude) |
1. Aplastic crisis (due to parovirus B19)
2. Autosplenectomy (Howell-Jolly bodies)-increased risk of infection with encapsulated organism 3. Splenic sequestration crisis 4. Salmonella osteomyelitis 5. Painful crisis (vaso-occlusive): Dactylitis (painful hand swelling, bone infarction), acute chest syndrome, avascular necrosis 6. renal papillary necrosis, and microhematuria (medullary infact) |
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Aldesleukin interleukin2
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renal cell carcinoma, metastatic melanoma
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EPOetin alfa EPO
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anemia esp in renal failure
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Filgrastim
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(G-CSF)
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Sargramostim
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GM-CSF
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alfa interferon
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Hepatitis B, C, Kaposi's sarcoma, leukemia, melignant melanoma
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beta interferon
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MS
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gamma interferon
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chronic granulomatous diseases
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oprelvekin interleukin-11
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thrombocytopenia
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Thrombopoietin
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thrombocytopenia
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Bleomycin: mechanism, clinical use and tox
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induce free radical formation, which cause DNA break,
testicular cancer, hodgkin's lymphoma PULMONARY FIBROSIS, skin hyperpigmentation, minimal myeosuppression |
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Etoposide, mechanism, clinical use and tox
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inhibit topoisomeraseII
solid tumors, leukemia, lymphoma myelosuppression, GI irritataion, alopecia |
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Cisplatin, mechanism, clinical use and tox
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crosslink DNA
testicular, bladder, ovary, lung Nephrotoxicity and acoustic nerve damage. Prevent kidney problem with amifostine (free radical scavenger) and chloride diuresis, (mannitol?) |
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thrombotic thrombocytopenic purpura TTP
PT, BT, mechanism lab symptom |
PC<, BT>
ADAMTS13 deficiency vWF metalloprotease, reduced degragation of vWF multimers. large vWF multimers-platelet aggregation and thrombosis, -platelet survival lab: schistocytes, LDH> symptom: pentad of neurological and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia |
