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Hematology - oncology Flashcards

1
Q

“Starry sky” lymph nodes: which disease? prevalence in which ages? genetic mutation? Viral association lesion常见部位？

A

Burkitt lymphoma (non-hodgkin lymphoma of mature B cells) occurs in adolescents or young adults genetic mutation: t (8: 14) [c-myc (8) and heavy chain Ig (14), - constitutive overproduction of C-Myc] Viral association - EBV lesion常见部位: in endemic form in Africa: jaw lesions; in sporadic form: pelvis or abdomen

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2
Q

2 direct factor Xa inhibitors? clinical use? 是否需要监测？toxicity?

A

Apixaban, rivaroxaban bind to directly inhibit Xa clinical use: 和warfarin类似，用于预防：treatment and prophylaxis of DVT and PE (rivaroxaban), stroke prophylaxis in pts. with AF 口服， 一般不需要监测 toxicity：bleeding, [no specific reversal agent available]

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3
Q

5-fluorouracil (5-FU) 是什么药？机制 （target which phase of cell cycle)？用途？toxicity以及如何reverse?

A

抗肿瘤 机制 : pyramidine analog bioactivated to 5F-dUMP, which covalently complexes with folic acid; this complex inhibit thymidylate synthase ⇒ ↓ dTMP ⇒ ↓ DNA and protein synthesis target which phase of cell cycle： s phase-specific 用途: colon cancer, pancreatic cancer, basal cell carcinoma (topical) toxicity以及如何reverse: 1. 类似MTX有myelosuppression, 但和MTX不一样（不能用leucovorin亚叶酸）reverse: overdose rescued with uridine 2. photosensitivity

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4
Q

ACD (anemia of chronic disease)引起贫血的机理？Lab? 属于哪种贫血？

A

inflammation ⇒ liver release of hepcidin ↑ ⇒ binds ferroportin on intestinal mucosal cells and macrophages to inhibit iron transport ⇒ ↓ release of iron from macrophage Lab: ↓ iron, ↓ TIBC, ↑ ferritin 一般是nonhemolytic, normocytic, 也可以是microcytic, hypochromic

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4
Q

Acute intermittent porphyria: affect E? accumulated substances? Cx? Rx?

A

affect E: porphobilinogen deanimase accumulated substances: porphobilinogen, delta-ALA, urine coporphobilinogen Cx: 5 P’s Painful abdomen Port wine-colored urine Polyneuropathy Psychological disturbances Precipitated by drugs, alcohol, starvation Rx: glucose + heme (inhibit ALA synthase)

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4
Q

Abciximab是什么药？机制？用途？toxicity

A

抗凝，inhibit platelet aggregation by binding to GIIb/IIIa on activated platelets made from McAb Fab fragments 用途: unstable angima, PTCA (经皮穿刺冠状动脉造影） toxicity: bleeding, thrombocytopenia

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5
Q

adult T-cell lymphoma: 发病年龄? specifically affect which populations? virus association? Cx?

A

发病年龄: adults specifically affect which populations: Japan, West Africa, Carribbean virus association: HTLV-1 (Human T-cell lymphotropic virus type 1), IV drug abuse Cx: lytic bone lesions, hypercalcemia

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6
Q

ALL: age? associated with other disease? Peripheral blood and BM see? Cx? Surface markers? does it respond to therapy? which gene translocation has better prognosis?

A

age: < 15 associated with other disease - downs Peripheral blood and BM see: ↑↑↑ lymphoblasts Cx- 如果是T cell ALL, 可表现为纵膈mass (infiltrate thymus) Surface markers: CD10+ (if pre-B cell only); TdT+ (pre-T and pre-B both) does it respond to therapy - yes which gene translocation has better prognosis: t (12:21)

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6
Q

AML: age? Peripheral blood smear? risk factors? genetic mutation？ Rx? 常见的一个Cx?

A

age: median onset of 65 Peripheral blood smear: 1) 非常特征性的Auer rods (奥尔小杆:白血病患者成髓细胞胞浆中小杆状体）， 2) peroxidase+ cytoplasmic inclusions seen mostly in M3 AML; 3) ↑↑↑ circulating myeloblasts risk factors: alkylating chemotherapy, radiation, myeloproliferative disorders, downs genetic mutation: t (15:17) - M3 Rx:所有AML 亚型都respond to AT-RA (VitA), which induces differentiation of myeloblasts 常见的一个Cx: DIC (can be induced by chemotherapy due to release of Auer rods)

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7
Q

Argatroban（阿戈托班）, bivalirudin是什么药？

A

抗凝血药（derivatives of hirudin 水蛭素）-inhibit thrombin directly used instead of heparin for anti-coagulating pts with HIT

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8
Q

associated patho basophilic stippling?

A

Basically, ACiD alcohol is LeThal. Basophilic stippling: anemia of chronic disease, alcohol abuse, Lead poisoning, Thalassemias

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9
Q

associated patho: acanthocyte?

A

liver disease, abetalipoproteinemia (cholesterol dysregulation)

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9
Q

associated patho: teardrop RBC Target cell

A

teardrop RBC - BM infiltration (RBC sheds a tear because it’s forced out of its home) Target cell: “HALT”, said the hunter to his Target (HbC disease - beta-globin mutation引起的贫血, asplenia, Liver disease, Thalassemia

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10
Q

associated patho: Heinz bodies

A

oxidation of Hb sulfhydryl group (硫氢基) → denatured Hb precipitation and phagocytic damage to RBC membrane → bite cells crystal violet可以把Heinz bodies染出来 seen in G6PD deficiency, Heizn body-like inclusions seen in a-thalassemia

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10
Q

associated patho: Howell-Jolly bodies

A

脾脏是个吞噬RBC的器官，嚎叫着（howell)吃Jelly (Jolly) Howell-Jolly bodies: basophilic nuclear remnants found in RBC, normally removed by splenic macrophages seen in functional hyposplenia, or asplenia

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12
Q

basophil function? granules contain? Isolated basophilia indicates?

A

basophil function - mediate allergic reaction granules contain: 1) heparin (anticoagulant) 2) histamine (vasodilator) 3) leukotrienes Isolated basophilia indicates myeloprolifeative diseases, especially CML

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13
Q

bevacizumab 什么药？mechanism? clinical use? toxicity?

A

北伐单抗 mechanism： McAb against VEGF ⇒ inhibit angiogenesis clinical use： solid tumors (colorectal cancer, renal cell carcinoma) toxicity: hemorrhage and impaired wound healing

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14
Q

bleomycin: 是什么药？机制 ？用途？toxicity:

A

博来霉素，抗肿瘤药 机制: induces free radical formation, to cause breaks in DNA strands (G2) 用途: tesicular cancer, Hodgkin lymphoma toxicity: 1) pulmonary fibrosis!! 2) skin changes, mucositis note: 和别的抗代谢药不一样：minimal myelosuppression

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15
Q

Cilostazol, dipyridamole是什么药？机制？用途？toxicity?

A

Cilostazol (西洛他唑）, dipyridamole （双嘧达莫; 潘生丁）：抗凝药 机制：PDE III inhibitor: 1) ↑ cAMP in platelets, thus inhibit platelet aggregation; 2) vasodilators 用途: intermittent claudication (PAD的上佳药物）, coronary vasodilation, presentation of stroke or TIAs when combined with aspirin, angina prophylaxis toxicity: 因为扩张血管引起：nausea, headache, facial flushing, hypotension, abdominal pain

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16
Q

cisplatin, carboplatin: 是什么药？机制 ？用途？toxicity以及如果预防？

A

anti-tumor 机制: cross link DNA 用途: testicular, ovary, bladder, and lung carcinoma toxicity： 1）nephrotoxicity (prevented with amifostine 阿米斯丁， free radical scavenger) and chloride diuresis 2) acoustic nerve damage

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17
Q

CLL/SLL （small lymphocytic lymphoma): age? Peripheral blood smear? Cx? Surface markers? SLL和CLL鉴别？

A

age: > 60 Peripheral blood smear: “smudge cells” Cx: often无症状，进展很缓慢 Surface markers：CD20+/CD5+ B-cell neoplasm SLL和CLL鉴别: same as CLL except CLL has ↑ peripheral blood lymphocytosis or BM involvement

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18
Q

CML: age? Peripheral blood smear? risk factors? genetic mutation？ E的变化？ Cx? Rx?

A

age: 45-85, median 64 Peripheral blood smear: ↑ neutrophils, metamyelocytes, basophils genetic mutation: Philadelphia chromosome ( t (9:22), bcr-abl) E的变化: very low LAP (leukocyte alkaline phosphatase) (vs. leukemoid reaction, in which LAP ↑) Cx: 肝脾肿大，may transform to AML or ALL (blast crisis) Rx: imatinib (small-molecule inhibitor of the bcd-abl tyrosine kinase)

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19
Q

CO poisoning对O2 delivered to tissue有什么影响？ 这些指标怎么变化：PaO2? carboxyhemoglobin? methemoglobin?

A

CO poisoning对O2 delivered to tissue有2大影响： 1. 对Hb有超过O2 250倍的affinity, 占据Heme位点后，O2无法再和heme结合 ⇒ O2 content of blood ↓ (这一点和hypoxia一样） 2. 使Hb-O2 dissociation curve left shift!!! [非常重要的变化，意味着即使氧气好不容易结合上血红蛋白被运送到了组织，也很难被释放出来 - 这是一般hypoxia中没有的变化】 CO中毒时： PaO2： 不变（这是partial pressure of O2 dissolved in the plasma, 是氧气的溶解度，和CO中毒一点关系都没有） carboxyhemoglobin：↑ methemoglobin 高铁血红蛋白：不变（这是由Fe2+被氧化为Fe3+时升高，出现在nitrites, dapsone【麻风病中的抗生素】中毒时）

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20
Q

corticosteroids对neutrophil, eosinophil, lymphocytes的影响？

A

corticosteroids对neutrophil的影响：cause neutrophilia 中性白细胞增多, 但是这些中性粒到不了感染部位：corticosteroids ↓ activation of neutrophil adhesion molecules, impair their migration out from the vasculature to the site of inflammation corticosteroids对eosinophil的影响: eosinopenia [sequester eosinophils in lymph nodes] corticosteroids对 lymphocytes的影响: lymphopenia [cause apoptosis of lymphocytes

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21
Q

Cytarabine 是什么药？机制 （target which phase of cell cycle)？用途？toxicity

A

pyrimidine analogs ⇒ inhibit DNA polymerase taget S-phase (和MTX, 5-FU不同的地方是：与叶酸代谢无关） 用途： lymphoma, leukemias toxicity: CYTarabine causes panCYTopenia leukapenia, thrombocytopenia, megaloblastic anemia

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21
Q

dactinomycin: 是什么药？机制 ？用途？toxicity

A

放线菌素D = actinomycin D 机制: intercalates in DNA 用途: childhood tumors (“children act out”) Wilms tumor, Ewing sarcoma (10 - 20 yrs old, sarcoma in pelvis or long tubular bones); rhabdomyoscarcoma 横纹肌肉瘤 toxicity: myelosuppression

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21
Q

cyclophosphamide, ifosfamide(IFO): 是什么药？机制 ？用途？toxicity (如何预防）？

A

alkylating agents (anti-tumor) 烷化剂 机制：covalently X-link (inter strand) DNA at guanine N-7; [require liver bioactivation] 用途: solid tumors, leukemia, lymphomas, some brain cancers toxicity (如何预防）： 1) myelosuppresson 2) hemorrhagic cystitis - partially prevented with mesna [美司钠,巯乙磺酸钠: its thiol group binds toxic metabolites]

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23
Q

define: dendritic cells? function? surface marker? what is it called in the skin?

A

dendritic cells function - highly phagocytic APCs; link innate and adaptive immune systems surface marker: MHC II, Fc receptor what is it called in the skin - Langerhans

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23
Q

DIC: causes? Labs?

A

causes: “STOP Making New Thrombi” (widespread activation of clotting - deficiency in clotting factors, - a bleeding state S: sepsis (gram-negative) Trauma Obstetric complications (例如羊水栓塞） acute Pancreatis Malignancy Nephrotic syn Transfusion Labs: schistocytes, ↑ D-dimer (fibrin split products), ↓ fibrinogen, ↓ factors V and VIII. ↓ PC, ↑ BT, PT, PTT

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23
Q

define: tumor stage? grade?

A

tumor stage: how far the tumor spread to the adjacent tissue, lymph nodes, metastasis, grade: differentiation

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24
Q

Down syn pts: 儿童期死因？成人死因？

A

儿童期死因：acute lymphoblastic (or myelogenous) leukemia 成人死因: AD

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25
Q

doxorubicin: 是什么药？机制 ？用途？toxicity: which drug to prevent the toxicity?

A

阿霉素, 羟基柔红霉素 【抗肿瘤药】 机制: 1) intercalates in DNA ⇒breaks in DNA ⇒ ↓ replication (for G2 phase) 2) generate free radicals 用途: solid tumors, leukemias, lymphomas toxicity: 1) cardiotoxicity - dilated cardiomyopathy - prevented by Dexrazoxane 右旋丙亚胺(主要用于保护阿霉素引起的心脏毒性) 2) myelosuppression 3) alopecia 脱发 4）toxic to tissues following extravasation 外渗

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26
Q

Dx: neutrophils with bilobed nuclei (2 nuclear masses connected with a thin filament of chromatin)?

A

Pelger-Huet anomaly, or Pseudo Pelger-Huet anomaly Pseudo Pelger-Huet anomaly: typically seen after chemotherapy 真性的话是良性genetic disorder (AD)

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27
Q

eptifibatide是什么药？机制？用途？toxicity

A

依替巴肽 和abciximab，tirofiban 一类, inhibit platelet aggregation by binding to GIIb/IIIa on activated platelets 用途: unstable angima, PTCA (经皮穿刺冠状动脉造影） toxicity: bleeding, thrombocytopenia

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28
Q

etoposide是什么药？机制？用途？toxicity

A

VP-16, 依托泊苷,鬼臼毒素(抗肿瘤药) 机制: inhibit topoisomerase II (target cell cycle S and G2 phases) 用途？toxicity

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28
Q

etoposide, teniposide 是什么药？机制 ？用途？toxicity以及如果预防？

A

VP-16, 鬼臼毒素 替尼泊苷; 鬼臼噻吩苷 机制: Etoposide inhibits DNA topoisomerase II - ↑ DNA degradation 用途: 1. solide tumors (particularly testicular and small cell lung cancer) 2. leukemias, lymphoma toxicity： 1. myelosuppression 2. GI irratation 3. alopecia

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29
Q

G6PD deficiency是什么遗传？病因？lab? Cx?

A

[Stress makes me eat bites of fava beans with Heinz ketchup] X-linked recessive (XR) MC RBC酶缺陷 defect in G6PD ⇒ ↓ glutathione ⇒ ↑ RBC susceptibility to oxidative stress 病因: 增加oxidative stress的因素：Fava beans!!， sulfa drugs, antimalaries, infections Cx: oxidative stress后几天出现back pain, hemoglobinuria Lab: blood smear shows RBCs with Heize bodies and bite cells

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31
Q

Glanzmann thrombasthenia 血小板机能不全 发病机理？能被哪个抗凝药物模拟？

A

deficiency in GpIIb/IIIa Cx: childhood muccotaneous bleeding Dx: peripheral smear shows no platelet clumping = abciximan (inhibit GpIIb/IIIa directly)

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32
Q

Hairy cell leukemia: B or T cells? age? Peripheral blood smear? Dx? 骨穿症状？ Rx?

A

mature B-cell tumor age: elderly Peripheral blood smear: hair-like projections Dx: 不再用FLOW, 改用TRAP诊断 (tartrate-resistant acid phosphatase + ) 骨穿症状: marrow fibrosis, dry tap on aspiration Rx: Cladribine (2-CDA) 克拉屈滨,2-氯脱氧腺苷, an adenosine analog to inhibit ADA (adenosine deaminase)

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33
Q

heme synthesis中得限速酶？associated disease?

A

ALA synthase (delta-aminolevulinic acid) associated disease: sideroblastic anemia (X-linked)

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34
Q

Hemophilia A/B: deficiency of which factor? coagulation pathway? vWF carries/protects?

A

Hemophilia A: deficiency of factor VIII Hemophilia B: deficiency of factor IX vWF carries/protects VIII

35
Q

heparin抗凝的原理？

A

heparin enhances the activity of antithrombin antithrombin inhibits activated forms of factors II, VII, IX, X, XI, XII

36
Q

heprain: mechanism? Difference with LMWH? half life? Clinical use? Monitor? Diff with LMWH? Complications - what is the most severe one, how it’s developed? Antidote for Heparin（how it works)? 如果发生HIT又要抗凝，用什么药？

A

heprain: cofactor for antithrombin; ↓ thrombin, ↓ factor Xa LMWH: ↓ factor Xa (缺少长链肝素的5肽，无法bind住antithrombin-thrombin complex) half life - short, so used as “immediate anticoagulation” in PE, acute coronary syn, MI, DVT; do not cross placenta - used in pregnancy Monitor: PPT LWMH (enoxaparin, delteparin): act on Xa, better bioavailability, can be used sc, 2-4 times long half-life; 不用monitor Complications - HIT; bleeding, osteoporosis, drug-drug interactions what is the most severe one, how it’s developed: HIT (development of IgG Ab against heparin bound to platelet factor 4; Ab-heparin-PF4 complex activates platelets ⇒ thrombosis and thrombocytopenia Antidote （how it works): protamine sulfate (negatively charged heparin binds to positively charged protamine) 如果发生HIT又要抗凝，用什么药：argatroban （derivatives of hirudin 水蛭素）-inhibit thrombin directly

38
Q

Hereditary spherocytosis [HS, 遗传性球形红细胞增多症] 会引起aplastic crisis, 这是什么原因？ lab? Rx?

A

Hereditary spherocytosis会引起aplastic crisis - parvovirus B19 细小病毒 infection lab: 诊断: osmotic fragility test +; screen: eosin-5-maleimide binding test 【 Flow cytometric analysis of eosin-5-maleimide (EMA)-labeled red blood cells (RBCs) has been used as a screening test】 Rx：splenectomy

39
Q

HUS (hemolytic uremic sym 溶血性尿毒症）： 好发人群？cause? Cx? lab?

A

好发人群：children < 10-yr cause： EHEC O157:H7 - secret Shiga-like toxin (ingest undercooked beef, drink unpasteurized milk or contaminated water, person-to-person contact) Cx: 血性腹泻 + 肾衰 （oliguria, ↑ BUN+creatinine ) + 严重腹痛 + CNS syn (somnolence, lethargy) Lab: peripheral blood smear shows fragmented RBCs (UW还以conjuctival pallor的方式考过贫血) 看这个病的名字就知道临床表现：溶血+尿毒症！

40
Q

hydroxyurea: 机制 ？用途？toxicity？

A

机制: inhibits ribonucleotide reductase - ↓ DNA Synthesis (S-phase specific) 用途: 1. melonoma 2. CML 3. sickle cell anemia (↑ HbF) toxicity： 1. BM suppression 2. GI upset

41
Q

Langerhans cell histocytosis (组织细胞增多症） cause? Cx? Cell markers? Dx?

A

cause: proliferative disorder of dendritic (langerhans) cells from the monocyte lineage; cells are immature and can’t stimulate primary T cells via antigen presentation Cx: child, lytic bone lesions, skin rash or recurrent otitis media, with a mass in the mastoid bone颞骨乳突 Cell markers: S-100 (mesodermal origin)+ /CD1a + Dx: EM 见特征性Birbeck granules (tennis rackets)

41
Q

irinotecan, topotecan: 是什么药？机制 ？用途？toxicity？

A

anti-tumor 机制: inhibits DNA topoisomerase I - prevent DNA unwinding and replication 用途: 1. irinotecan: colon cancer 2. topotecan: ovary and small cell lung cancer toxicity： 1. severe myelosuppression 2. diarrhea

41
Q

Imatinib (Gleevec): mechanism? clinical use? toxicity?

A

伊马替尼 mechanism: 1) Tyr kinase inhibitor of bcr-abl (Philadelphia chromosome fusion gene in CML) 2) Tyr kinase inhibitor of c-kit (common in GI stromal tumors) clinical use: CML, GI stromal tumors toxicity: fluid retention

42
Q

Lead poisoning: Lead inhibits which enzymes? 后果？ children/adults 最常见的exposure是什么？ Cx? Lab? Dx?

A

Lead 1) inhibits ferrochelatase (亚铁螯合酶） and ALA dehydratase ⇒ ↓ heme synthesis, ↑ RBC protoporphyrin 2) inhibits RNA degradation ⇒ RBCs retain aggregates of rRNA (basophilic stippling 噬碱性点彩） Cx: LEAD Lead Lines on gingival, on metaphases of long bones on X-ray Encephalopathy and Erythrocyte basophilic stippling Abdominal colic and sideroblastic Anemia Drops - wrist and foot drop children - exposure to lead paint ⇒ mental deterioration adults - environmental exposure (battery/radiator/aummunition 弹药）factory ⇒ headache, memory loss, demyelination Lab: ↑ blood protoporphyrin, delta-ALA Dx: 1st line: Dimercaprol 二硫基丙醇, EDTA; succimer二硫琥珀酸在kids中用做螯合剂（it sucks to be a kid who eats lead)

44
Q

leukemoid reaction 的lab? 如何与CML鉴别？

A

leukemoid reaction： acute inflammatory response to infection lab：↑ WBC count with ↑ neutrophils and its precursors (band cells left shift) ; ↑ leukocyte ALP 如何与CML鉴别: CML also ↑ WBC count with left shift; but ↓ leukocyte ALP !!!

45
Q

Macrocytic anemias除了巨细胞性贫血外的病因？

A

diseases that DNA synthesis is impaired： liver disease, alcoholism, drugs: 5-FU, zidovudine (AZT), hydroxyurea

47
Q

Mantle cell lymphoma: mutation? 发病年龄? B cell marker?

A

t (11:14) translocation of Cyclin D1 (11) and heavy-chain Ig (14) in older males, CD5+

48
Q

mast cells: bind to Fc of which Ig? cross-linking cause? involved in which type of hypersensitivity? mechanism for cromolyn sodium in asthma prophylaxis?

A

bind to Fc of IgE IgE cross-linking cause release of histamine, heparin, eosinophil chemotactic factors involved in type I of hypersensitivity mechanism for cromolyn sodium in asthma prophylaxis - prevent mast cell degranulation

50
Q

MC porphyria? affected E? accumulated substances? Cx?

A

MC porphyria - porphyria cutanea tarda 迟发型皮肤卟啉症 affected E - uroporphyrinogen decarboxylase accumulated substances- uroporphyrin (tea-colored urine) Cx - blistering cutaneous photosensitivity

51
Q

methotrexate (MTX) 是什么药？机制 （target which phase of cell cycle)？用途？toxicity以及如何reverse?

A

甲氨喋呤 机制- folic acid analog that inhibits dihydrofolate reductase ⇒ ↓ dTMP ⇒ ↓ DNA (both purines and thymidine) and protein synthesis S-phase specific 用途: 2大临床应用：肿瘤及非肿瘤 1. cancers: leukemias, lymphomas, choriocarcinoma (绒毛膜癌), sarcomas 2. non-neoplastic: abortion, ectopic pregnancy (drug of choice in < 6 wk宫外孕） autoimmune类（RA-治疗风湿性关节炎一线药物，UW考到在RA病人中引起肝脏毒性； psoriasis - UW考到治疗牛皮癣的机制是target rapid proliferating epithelial cells in the skin, IBD) toxicity: 1. 骨髓毒性 myelosuppression: reversible with Leucovorin 亚叶酸, “folinic acid” [甲氨蝶岭解毒药,抗贫血药] 2. 肝脏毒性：macrovesicular fatty change; 长期服用MTX (例如RA)可见cirrosis 3. mucositis 粘膜炎 4. teratogenic

52
Q

direct vs. indirect Coombs test?

A

direct: 往病人RBC中加入anti-Ig Ab (Coombs reagent). 如果病人RBC表面coated with Ig, 就会凝聚。 Indirect: 取病人serum加入正常RBC. 如果病人血清中含有anti-RBC surface Ig, RBC就凝集。

53
Q

monocytes - macrophages : 各自存在什么地方？ macrophages activated by which cytokine? function as APC via? surface marker?

A

monocytes - circulating in the blood macrophages : in tissues macrophages activated by which cytokine? -INFg function as APC via MHC II surface marker CD14

54
Q

Multiple Myeloma (MM): 病理特征细胞？ associated with? (x-ray, 电泳，urine, RBC) Cx? 鉴别：1）Waldenstrom macroglobulinemia; 2) MGUS (monoclonal gammopathy of undetermined significance)

A

病理特征细胞: numerous plasma cells with “clock face” or “fried egg” appurtenance: large eccentric nuclei, intracytoplasmic inclusions containing Ig [produce large amounts of IgG (55%) or IgA (25%)] associated with: 1. ↑ susceptibility to infection 2. AL (primary amyloidosis) 3. X-ray: punched-out lytic none lesions 4. Serum protein electrophoresis: M spike (和下面2个病鉴别） 5. urine: Ig light chain (Bence Jones protein) 6. RBC: rouleaux formation on smear Cx: CRAB is yuMMy!! hyperCalcemia (溶骨） Renal insufficiency Anemia Bone lytic lesions/Back pain Multiple Myeloma： Monoclonal, M protein spike M spike还见于以下2个病，需要鉴别： 1. Waldenstrom macroglobulinemia； 1) M spike = IgM (not IgG or A in MM) 2) no lytic bone lesions) 2) MGUS (monoclonal gammopathy of undetermined significance): asymtomatic precursor of MM M spike

55
Q

mycosis fungoides/Sezary syn 蕈样霉菌病/塞泽瑞(氏)综合征 是一种什么疾病？ 发病年龄? Cx? cell surface marker?

A

neoplasms of mature T cells 发病年龄: adults Cx: cutaneous patches/plaques, potential to spread to lymph nodes and visera 【most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. 】 cell surface marker: circulating malignant cells CD4+, indolent (无痛的）

56
Q

myelofibrosis: define mutation? BM改变？

A

fibrotic obliteration of BM 30~50% have JAK2 mutation Teardrop RBCs and immature forms of the myeloid line “BM is crying because it’s fibrosed”

57
Q

Neutrophil: function? major chemotaxic factor for neutrophils? 形态？异常形态？ 2种granules各含什么酶？ ↑ band cells意味着什么？

A

function: acute inflammatory response cell, ↑ in bacterial infection 1) chemotaxis; 2) phagocytosis major chemotaxic factor for neutrophils: IL-8 released by macrophage 形态 - multilobed nucleus 异常形态- hypersegmented polys ( > 5 lobes), seen in VB12 / folate deficiency 2种granules各含什么酶: 1) small, more numerous granules: ALP, collagenase, lysozyme, lactoferrin 2) large, less numerous azurophilic 嗜苯胺蓝颗粒 granules: = lysosomes, contain proteinases, acid phosphatase, myeloperoxidase, beta-glucuronidase ↑ band cells (immature neutrophil) 意味着什么- reflects ↑ myeloid proliferation = bacterial infection, CML

58
Q

nitrite poisoning机制？表现？ 这些指标怎么变化：PaO2? O2 content of the arterial blood? O2 carrying capacity of the arterial blood? bound fraction of O2 in the arterial blood (= % saturation)? Rx?

A

机制： nitrites oxidize the heme iron from Fe2+ (ferrous state) to Fe3+ (ferric), 形成methemoglobin 高铁血红蛋白， which CANNOT bind to O2, and LEFT SHIFT the Hb-O2 dissociation curve (这两点和CO poisoning一样） 表现： 1. cyanosis that cannot be corrected by O2 supplementation, 2. functional anemia (weakness, dyspnea, headaches, etc) PaO2 - normal O2 content of the arterial blood: ↓ O2 carrying capacity of the arterial blood ↓ bound fraction of O2 in the arterial blood (= % saturation) ↓ Rx: methylene blue

59
Q

nitrosoureas: 亚硝尿类 包括哪些？是什么药？机制 ？用途？toxicity？

A

carmustine 卡莫斯丁 lomustine 罗莫斯丁 (环己亚硝脲) semustine 司莫司汀 (甲环亚硝脲) streptozocin 链佐星，琏唑霉素 抗肿瘤药，require bioactivation 机制: cross link DNA, cross BBB 用途: brain tumors, including GBM toxicity: CNS toxicity (convulsions, dizziness, ataxia)

60
Q

O2-Hb dissociation curve什么情况left shift? right shift?

A

left shift: 氧气难以从血红蛋白释放出来 ↓ H+ (= pH上升，血液偏碱性） ↓ 2，3DPG ↓ temperature (低温会稳定O2和Hb结合的化学键，让O2难以释放） right shift：氧气容易从血红蛋白释放出来 ↑H+ (= pH上升，血液偏碱性） ↑ 2，3DPG ↑ temperature (高温时身体需氧）

61
Q

paroxysmal nocturnal hemoglobinuria (PNH) 病因？lab? Cx? Rx?

A

病因: ↑ complement-mediated RBC lysis; aquired mutation in HSCs; ↑ incidence of acute leukamias lab: CD55/59 (-) RBCs on Flow Cx: triad (Coombs(-) hemolytic anemia, pancytopenia, VT [stem cell deficiency, 全血受累】 Rx： eculizumab 【mAb against complement. 第一个FDA批准治疗PNH的药，2011年第一个批准治疗aHUS (atypical hemolytic uremic sun]

62
Q

platelet: life span? contain which granules? receptors for each factors?

A

life span - 8-10 days contain which granules: 1. dense granules (ADP, calcium) 2. alpha granules (vWF, fibrinogen) receptor for vWF: GpIb receptor for fibrinogen: GbIIb/IIIa

64
Q

polycythemia vera： mutation? Lab? Cx? if 1°, EPO的变化？ If 2°, caused by ?

A

真性RBC增多症 mutation：JAK2 (mutation implicated in myeloproliferative disorders other than CML) Lab: hematocrit > 55%, ↑ RBC, WBC and platelets Cx: 最常见的presentation是intense itching after hot shower; 严重的（但是也很经典的）症状是erythromelalgia 红斑性肢痛症; due to episodic blood clots in vessels of the extremities if 1°, EPO ↓ If 2°, caused by : natural or artificial ↑ EPO

65
Q

prednisone, predisolone用于抗癌的机制？ clinical use?

A

prednisone, predisolone are the most MC glucocorticoids used in cancer therapy, 常是化疗联用药物。 用于抗癌的机制: tigger apoptosis; even work on nondividng cells!! clinical use: 1. CLL 2. non-Hodgkin lymphoma toxicity: 1. cushing-like symptoms: weight gain, central obesity, muscle breakdown, cataracts, acne, osteoporosis 2. HTN, peptic ulcers, 3. hyperglycemia 4. psychosis

66
Q

purine analog类抗代谢药：3大代表？ 机制: activated by which E? target which phase of cell cycle？用途？ toxicity? 在和allopurinol联用时会出现什么问题？

A
  1. azathioprine 硫唑嘌呤 2. 6-mercaptopurine (6-MP) 3. 6-thioguanine (6-TG) 机制: ↓ de novo purine synthesis; activated by HGPRT 用途: 1. prevent organ rejection, RA 2. SLE (azathioprine) 3. leukemia, IBD (6-MP, 6-TG) toxicity: 1. BM, liver, GI 毒性 2. azathioprine and 6-MP are metabolized by xanthine oxidase, thus both have ↑ toxicity with allopurinol （which inhibits their metabolism)
67
Q

rasburicase 什么药？用途？

A

拉布立酶 recombinant urate oxidase (present in mammals but not in humans) prevent and treat hyperuricemia and the resulting renal manifestations of tumor lysis syn

69
Q

Reed-Sternberg cells: marker for which disease? surface CD markers? MC type, 在男女两性中分布比例？ 决定预后的因素？

A

distinct tumor giant cells in Hodgkin lymphoma (owl eyes) CD15+/CD30+ B cell origin (2 owl eyes * 15 = 30) necessary but not sufficient for a Dx of Hodgkin disease MC type: nodular sclerosing form 在男女两性中分布比例: equally affected 决定预后的因素: lymphocyte-rich form has best prognosis; lymphocyte mixed or depleted forms have poor prognosis

70
Q

Retinoblastoma: Cx包括一个特征reflex? if familial, MC secondary malignancies:

A

特征reflex: white pupillary reflex (leukocoria 白瞳〔症〕，瞳孔泛白) if familial, MC secondary malignancies: osteosarcoma

72
Q

Reye Sym Cx? Lab? microscope finding?

A

use of aspirin in 5-14 yr old children with viral infection (aspirin should be avoided for children < 16, 除非是Kawasaki disease) multiple organ damage, especially brain and liver rash, vomiting, and liver damage Lab: 1) hepatic failure: ↑ ALT, AST, ammonia, bilirubin, PT and PPT microscope: microvesicular steatosis (肝脏细胞中脂肪小粒） 2）encephalopathy: ↑ ammonia from liver failure causes cerebral edema

73
Q

ristocetin作用机理？

A

activate vWF to bind to GpIb (促进凝血）

74
Q

rituximab： 什么药？mechanism? clinical use? toxicity?

A

利妥昔单抗 mechanism: McAb against CD20 (surface marker for most B cell neoplasms) clinical use: 1. non-Hodgkin lymphoma 2. 和MTX联用治疗RA 3. ITP toxicity: ↑ risk for progressive multifocal leukoencephalopathy

75
Q

sickle cell anemia: 病因？lab? 成人中最凶险的一个并发症？Dx? Rx?

A

病因：HbS point mutation ⇒ b chain position 6: glutamic acid → Val mutation lab： crescent-shaped RBC, “crew out” on skull X-ray (BM expansion, 和thalassemias颅骨表现一样） 成人中最凶险的一个并发症: acute chest sun ( vaso-occlusive crisis of the pulmonary vasculature commonly seen in patients with sickle cell anemia. This condition commonly manifests with pulmonary infiltrate on a chest x-ray) Dx: Hb electrophoresis Rx: HU (to ↑ HbF) and BMT

76
Q

sideroblastic anemia有哪些病因？ lab? Dx?

A

病因: defect in heme synthesis 1). genetic： X-linked, defect in delta-ALA synthase gene 2) aquired: myelodysplastic syndrome 3) reversible: MC is Alcohol, VB6 deficiency, copper deficiency, isoniazid Lab: 1) BM 见ringed sideroblasts, with iron-laden mitochondria 2) ↑ iron, ↑ ferritin, normal TIBC Dx: pyridoxine (B6, a co-factor for delta-ALA synthase)

77
Q

t (14:18)导致什么基因表达上升？导致哪两种疾病？ 各种发病年龄？

A

translocation of heavy-chain Ig (14) : bcl-2 (18), 导致overproduction of Bcl-2 (which inhibits apoptosis) 出现于1）diffuse large B-cell lymphoma; 2) Follicular lymphoma 1. diffuse large B-cell lymphoma: usually older adults (MC type of non-Hodgkin lymphoma in adults), 20% in children 2。 Follicular lymphoma adults, bcl-2 inhibits apoptosis; presents with painless “waxing and waning” lymphadenopathy

77
Q

tamoxifen, raloxifene在cancer中用途？两者不同？ toxicity?

A

SERMs (selective ER modulators): antagonists in breast, agonists in the bone!!! clinical use: 1. tamoxifen: breast cancer treatment and prevention 2. raloxifene: 不能用于乳腺癌治疗，但可以用于预防；also used to prevent osteoporosis toxicity: 1. tamoxifen: ↑ risk for endometrial cancer (partial agonist in endometrium); hot flashes 2. raloxifene: no risk for endometrial cancer (antagonist in endometrium)

79
Q

taxols: 是什么药？代表？机制 ？用途？toxicity？

A

紫杉酚, anti-tumor (microtubule inhibitor) 代表: paclitaxel 紫杉醇 机制：hyper stabilize polymerized microtubules in M phase ⇒ mitotic spindle CANNOT break down (M-phase specific) “It is taxing to stay polymerized” 用途: ovarian and breast carcinomas 【女性喜欢用植物制品】 toxicity: myelosuppression, alopecia 脱发，hypersensitivity

81
Q

Thalassemia: 属于哪种贫血？ alpha-: gene defect? prevalence? findings: 4 alleles deletion? 3? 1-2? beta- : gene defect? prevalence? findings for minor? Major?

A

Thalassemia: 属于microcytic, hypochromic 贫血 a-: gene defect - a-globin gene deletion ⇒ ↓ a-globin synthesis prevalence: cis deletion in asian, trans deletion in African populations findings: 4 alleles deletion: no a-globin, not compatible with life, cause hydrous fetalis - 形成的globin Ɣ4 (“Hb brats”) 3 alleles deletion: HbH disease, very little a-globin, excess b-globin forms b4 (HbH) 1-2 alleles deletion: no clinically significant anemia b- : gene defect: point mutation in splice sites and promotor sequences ⇒ ↓ b-globin synthesis prevalence in Mediterrean population, 分为minor vs. major minor: heterozygote, b chain ↓, 通常无症状， Dx by ↑ HbA2 > 3.5% on electrophoresis Major: hemozygote, b chain is absent!! ⇒ severe anemia, require transfusion, can gain 2° hemochromatosis Marrow expansion⇒ skeletal deformities (“crew cut” on skull X-ray， “chipmunk” facies 髓外造血，肝脾肿大， ↑ risk of parvovirus B19-induced aplastic crisis ↑ HbF (a2Ɣ2). HbF is protective in infants, 在6个月后才开始起病 HbS/thalassemia heterozygote: mild to moderate sickle cell disease

82
Q

Ticlopidine, clopidogrel抗凝的原理？用于哪些情况？ Ticlopidine有一个严重的副作用？如何监测？

A

inhibit ADP-induced expression of GpIIb/IIIa， so to inhibit platelet aggregation used in percutaneous coronary intervention, unstable angina, non-Q wave MI ； 一般和aspirin联用， aspirin和clopidogrel是2个一线药。如果对它们过敏，可以用ticlopidine, 但是这个药在1%病人中引起严重的副作用：neutropenia, 表现为fever and mouth ulcer. 因此头3个月内biweekly whole blood count

83
Q

tirofiban是什么药？机制？用途？toxicity

A

替罗非班 和abciximab，eptifibatide 一类, inhibit platelet aggregation by binding to GIIb/IIIa on activated platelets 用途: unstable angima, PTCA (经皮穿刺冠状动脉造影） toxicity: bleeding, thrombocytopenia

84
Q

Trastuzumab (Herceptin): mechanism? clinical use? toxicity?

A

曲妥单抗，群司珠单抗 mechanism： McAb against HER-2 (c-erbB2, a tyr kinase receptor). inhibit HER2-initiated cellular signaling and Ab-dependent cytotoxicity ⇒ help kill cancer cells over expressing HER2 (2 = tras2zumab) clinical use: HER-2 + breast or gastric cancer toxicity: cardiotoxicity (HEARTceptin damages the HEART)

85
Q

vemurafenib （Zelboraf）： 什么药？mechanism? clinical use?

A

vemurafenib= V600E Mutation of bRAF Enzyme inhibitor mechanism: B-Raf enzyme inhibitor developed Genentech for the treatment of late-stage melanoma. The name “vemurafenib” comes from V600E mutated BRAF inhibition. clinical use： metastatic melanoma

86
Q

vinca alkaloids: 包括哪些？是什么药？机制 ？用途？toxicity？

A

长春花属抗肿瘤药 2大代表药物：vincristine 长春新碱，Vinblastine 长春花碱 机制：bind to beta-tubulin to inhibit its polymerization into microtubulin ⇒ inhibit formation of mitotic spindle (M-phase specific) 用途: solid tumors, leukemias, lymphomas toxicity: Vincristine: 中枢+外周神经毒性 (areflexia, peripheral neuritis), paralytic ileus Vinblastine: blast BM (suppression)

87
Q

VitK在凝血中的作用？ Wafarin抗凝的原理？用于什么疾病？为什么有delay作用？监测？？ 为什么新生儿要给VK? VitK deficiency会有哪些factor decrease?

A

oxidized VK 在epoxide reductase作用下变成reduced VitK, which acts as a cofactor for precursors for II, VII, IX，X, C, S to become mature (VitK deficiency这些factor都降低） Wafarin inhibits epoxide reductase/ vitK-dependent carboxylation of glutamic acid residues of clotting factors 。 用于VTE, VitK-dependent factors have long half-life, so wafarin has a few days’ delay for its onset of therapuetic window. monitored by PT (president went to War), standerized by INR Neonates lack enteric bacteria, which produces VitK

88
Q

vWF disease: defects? Genetics? Cx/lab? Dx? Rx?

A

defects: 1. defect in platelet plug formation: ↓ vWF ⟹ defect in platelet-to-vWF adhesion 2. defect in intrinsic coagulation pathway: because vWF carry/protect factor VIII: ↓ vWF ⟹ normal or ↑ PTT, depending on severity Genetics: AD, MC inherited bleeding disorder Cx/lab: mild bleeding, PC/PT正常，↑ BT, normal or ↑ PTT Dx: “ristocetin cofactor assay (vWF : RCo assay): ↓ agglutination is diagnostic Rx: DDAVP (Desmopressin: 1-desamino-8-D-arginine vasopressin), a synthetic derivative of the antidiuretic hormone, vasopressin. 这个药已经使用25年，被extensively studied; Mechanism of DDAVP: release vWF stored in endothelium

89
Q

Wafarin (coumadin): mechanism? 代谢？ clinical use? 监测？ toxicity的表现？治疗？

A

mechanism：inhibit the 1) synthesis and 2) gamma-carbooxylation of VK-dependent clotting factors (II, VII, IX, X) and Protein C, S [extrinsic pathway] site of action: liver!! 代谢: cytochrome P450 pathway [drug-drug interaction多不胜数！】 clinical use：慢性病和预防（与肝素相反：acute) STEMI (ST Segment Elevation Myocardial Infarction), venous thromboembolism prophylaxis, prevention of stroke in AF [not in pregnancy since it crosses placenta! 和肝素相反】 监测：PT/INR toxicity的表现: bleeding, skin/tissue necrosis, teratogenic, drug-drug interaction 治疗: 1) for reversal of warfarin overdose: give VitK 2) for rapid reversal of severe warfarin overdose: fresh frozen plasma

90
Q

warm vs. cold agglutinin?

A

autoimmune hemolytic anemia中的概念： Warm agglutinin = IgG (warm weather is great) Cold agglutinin = IgM (cold ice-cream is yummy) 很多热/冷凝集引起的溶血都是原发性

91
Q

↑ ESR 在哪些情况出现？ decrease?

A

↑ ESR: 1) infection 2) autoimmune (SLE, RA, temporal arteritis) 3) malignant neoplasms 4) GI disease (ulcerative colitis) 5) pregnancy decreased ESR: 1) polycythemia 2) sickle cell anemia 3) CHF 4) microcytosis 5) hypofibrinogenemia

92
Q

临床上唯一一个重要的non-enveloped, ss-DNA virus是什么？引起哪些疾病？

A

parvovirus 细小病毒组 B19: 1. erythema infectious (the fifth disease) 传染性红斑, 第五病 - “slapped face” rash on face 2. aplastic crisis in sickle cell disease 3. hydrops fetalis

93
Q

各条chromosome上有什么重要基因？ 8？ 9? 11？ 14？ 18？ 22? 导致疾病： t (8:14) t(14:18) t (11:14） t (15:17) t (9:22)

A

各条chromosome上有什么重要基因？ 8 - c-myc 9: bcr 11: cyclin D1 14: heavy chain Ig 18: bcl-2 22: abl t (8:14): Burkitt lymphoma (↑ C-myc) t(14:18): diffuse large B-cell lymphoma; follicular lymphoma (↑ Bcl-2) t (11:14）：Mantle cell lymphoma (↑ Cyclin D1) t (15:17): M3 type of AML (which responds to VitA) t (9:22): Philadelphia chromosome (CML) Bcr-abl hybrid

94
Q

巨细胞贫血3大病因？ 各自表现？lab? Dx?

A
  1. Folate deficiency 2. Vit B12 (cobalamin) deficiency 3. orotic aciduria 1. Folate deficiency: Cause: malnutrition (alcoholism), malabsorption, anti-folate (抗叶酸代表药物：methotrexate甲氨喋呤，trimethoprim TMP 三甲氨苄啶-二氢叶酸类似物，phenytoin), ↑ requirement (pregnancy, hemolytic anemia) Cx/lab: - hypersegmented neutrophils, glossitis, ↑ homocysteine (这3点和VB12缺乏一样） - ↓ folate, normal methylmalonic acid (甲基丙二酸），NO neurological symptoms (这3点和VB12缺乏鉴别！） 2. Vit B12 (cobalamin) deficiency： Cause: insufficient intake (完全吃素）, malabsorption (Crohn disease), pernicious anemia, proton pump inhibitors, fish tapeworm (阔节裂头虫） Cx/lab: - hypersegmented neutrophils, glossitis, ↑ homocysteine (这3点和folate缺乏一样） - ↓ B12, ↑methylmalonic acid (甲基丙二酸）, neurological symptoms (这3点和folate缺乏鉴别！） 神经症状是由myelin合成需要B12引起，累及中枢和外周： 1）peripheral neuropathy: sensorimotor dysfunction 2) dorsal column (vibration/proprioception) 感觉通路 3) lateral corticospinal (spasticity) LMN 运动通路 4) dementia 3. orotic aciduria: defect in UMP synthase ⇒ failure to convert orotic acid to UMP (de novo pyrimidine synthesis); AR Cx: children 表现为巨细胞贫血 (hypersegmented neutrophils, glossitis)，但是无法由folate 或者B12纠正; ↑ orotic acid 需要和ornithine transcarbamylase deficiency鉴别：后者除了↑ orotic acid, 还合并hyperammonemia Rx: UMP to bypass the mutated synthase
95
Q

病人present hypercoagulability, 考虑inherited 疾病？ 尤其是给warfarin置换后出现skin and subcutaneous tissue necrosis< 考虑什么疾病？

A

FA cover 4大hereditary thrombosis sym: 1. Factor V leiden (mutated factor V resistant to degradation by protein C; MCC of inherited hyper coagulability in whites) 2. prothrombin gene mutation (mutation in 3’-UTR: ↑ prothrombin- ↑ plasma levels and venous clots 3. antithrombin deficiency (如果是遗传的：ho no effect on PT, PTT or thrombin time, but diminish the ↑ in PTT by heparin; 也可以是获得性：renal failure/nephrotic syn, antithrombin lost in urine - ↑ factors II and X 4. protein C or S deficiency (↓ inactivation of factors V and VIII， ↑ risk of thrombotic skin necrosis with hemorrhage following administration of warfarin) “Protein C Cancels Coagulation)

96
Q

病人大量输全血后手足发麻是什么引起的？

A

> 5-6 L within 24 hrs: citrate in the whole blood or packed RBC is a chelator for Calcium - 输血引起的低钙血症

97
Q

血细胞分类， WBC从多到少排序？

A
  1. RBC 2. WBC: 1) 有颗粒（粒系 - granulocytes）：neutrophil, basophil, eosinophil 2）无颗粒（髓系 -mononuclear cells）：lymphocytes (B cells, T cells, NK), monocytes (mature into macrophage) 3) 血小板 WBC从多到少排序： Neutrophils Like Making Everything Better
99
Q

贫血分类

A

按体积分（MCV） 1. microcytic (MCV < 80 fL) 小细胞贫血：【死了铁，SLATI] Sideroblastic anemia (高铁成红细胞性贫血, copper deficiency can cause it) Lead poisoning ACD (anemia of chronic disease, late stage) Thalassemias Iron deficiency (late stage) 2. Normocytic (MCV = 80 - 100 fL) 又分为non-hemolytic (reticulocyte count正常或↓）和hemolytic (reticulocyte count ↑）: non-hemolytic : AACID ACD (early stage) Aplastic anemia Chronic kidney disease Iron Deficiency (early) hemolytic: 外源性溶血（autoimmune, infection), 和内源性溶血（RBC membrane or E. deficiency, Sickle cell, etc) 3. macrocytic (MCV > 100 fL) 1) megaloblastic: Folate/VB12 deficiency, orotic aciduria 乳清酸尿症 2) non-megaloblastic: liver disease, alcoholism, reticulocytosis

100
Q

补体系统是怎么激活？bind to which portion of immunoglobinlin?

A

The classical complement cascade begins when C1 binds to 2 IgG or 2 IgM (IgM is more potent activator, 因为是五聚体） C1 binds to the Fc region of heavy chain, NEAR the hinge!!

101
Q

鉴别Hodgkin vs. Non-Hodgkin lymphoma: 1. 累及nodes? extranodal? 有无contiguous spread? 2. 病理特征细胞？ 3. 年龄分布？ 4. virus association? 5. syms?

A

Hodgkin: 1. 累及nodes: localized, single groups of nodes extranodal? - rare 有contiguous spread, stage is the strongest predictor of prognosis [prognosis is much better than non-Hodgkin’s] 2. 病理特征细胞 - RS (Reed-Sternberg cells, owl eyes, CD15+ CD30+) 3. 年龄分布: bimodal (young adult and > 55; more common in men except for nodular sclerosing type) 4. virus association - 50% with EBV 5. syms: constitutional “B” signs/sym: low grade fever, night sweats, weight loss [B symptoms refer to systemic symptoms of fever, night sweats, and weight loss which can be associated with both Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. The presence or absence of B symptoms has prognostic significance and is reflected in the staging of these lymphomas.] Non-Hodgkin lymphoma: 1. 累及nodes: multiple, peripheral nodes extranodal: common 无contiguous spread 2. 病理特征细胞: majority involves B cells 3. 年龄分布: 20-40 4. virus association: may associate with HIV and immunosuprression 5. syms: fewer constitutional signs/sym

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