BHD Block 2 Diseases - Heme Diseases PDF

Category Name Presentation Lab findings/ immunophenotype Morphology Biochemistry...

Category Name Presentation Lab findings/ immunophenotype Morphology Biochemistry Genetics Testing Treatment Pathology images Other Notes Other Path Images Platelet adhesion is impaired due to endothelium failure to produce normal amounts of vWf, reduced factor VIII (vWf is carrier protein for VIII in blood). Mucosal Desmopressin (increases vWf bleeding (nosebleeds, bleeding low vWF, low vWF release from Weibel-Palade with dental work, heavy menses, activity, low factor bodies of endothelial cells) or easy bruising, heavy bleeding VIII. PT normal, vWF antigen level, risocetin replacement via recominant vWF with surgery), ecchymoses, PTT normal to Type 1 and 3 -> deficient vWf; Autosomal dominant. cofactor activity, factor VIII or cryoprecipitate (blood product von Willebrand's disease purpura, petechiae elevated type 2 -> defective vWf chromosome 12 levels, multimer assay made from donated plasma) Blood smear shows Bernard Soulier mild thrombocytopenia Syndrome Impaired platelet adhesion with enlarged platelets Lack of platelet GPIb Inherited platelet function Glanzmann defects (primary) Thrombasthenia Impaired platelet aggregation Lack of platelet GPIIb/IIIa Spontaneous and deep bleeds, hemophilic arthropathy (recurrent joint bleeds lead to synovial PTT markedly Replacement via factor VIII hypertrophy and cartilage elevated, PT concetrates (plasma derived and Hemophilia A erosion) normal Deficiency of factor VIII X-linked recessive PT and PTT recombinant) or cyroprecipitate Intrinsic pathway Factor VII deficiency Uncommon. Mucusal bleeding Autosomal recessive Extrinsic pathway Factor X deficiency Rare. Mucosal and deep bleeds Autosomal recessive Common pathway Rare. Mucosal bleeding. Deep Factor V deficiency bleeds can occur Autosomal recessive Common pathway Factor II (prothrombin) Rare. Significant mucosal and deficiency deep bleeds Autosomal recessive Common pathway Factor I (fibrinogen) deficiency Congenital fibrinogen defects Autosomal recessive Common pathway if stable, hold warfarin and wait, Blockage of gamma admin vit K. If pt bleeding or needs carboxylation of coagulation a procedure, give factor zymogens causes a decrease in concentrates, protrombin complex Warfarin Usage Platelet coagulation is impaired Prolonged PT vitamin K dependent factors N/A concentrate, and plasma Heparin inactivates thrombin and activated factor X through an antithrombin dependent mechanism (directly enhancing Heparin Usage Platelet coagulation is impaired Prolonged PTT AT3 activity) N/A Protamine sulfate Inability to produce Vit K Prolonged PT first, dependent coagulation factors ( possibly prolonged II, VII, IX, and X) and protein C & Vitamin K deficiency Platelet coagulation is impaired PTT S N/A Prolonged PTT Pathologic, systemic, and PT, prolonged uncompensated activation of thrombin time, hemostatic deceased platelt mechanismssecondary to an count, decreased Can be caused by sepsis (gram underlying disorder. Once fibrinogen, Schistoctes (caused by - bacteria,trauma, cancer, OB coagulation factord are increased D- fibrinclots in small complications, vascular treat the underlying condition and Clotting Factor Deficiencies Disseminated Intraasclar overused, there is excessive dimers, increased blood vessels disorders, toxins, immuno supportive care only as needed for (secondary hemostatsis) Coagulation bleeding fibrin split products shredding RBCs) disorders N/A life threatening bleeding microangipathic hemolytic anemia Most common thrombophilia. FV Inherited Thrombophilia Hypercoaguability with lower resistant to breakdown by (Factor V Leiden) thrombotic risk activated protein C G20210A mutation in prothrombin gene associated Inherited Thrombophilia Hypercoaguability with lower with increased prothrombin (Prothrombin 20210) thrombotic risk activity Life threatening formation of blood clots in small vessels Caused by ADAMTS13 throughout the body absence/decrease (congenital or (microangiopathic hemolytic acquired, ADAMTS13 is a vWF anemia). fever, hemolyic anemia, cleaving protease. absence Thrombotic renal dysfunction, results in accumulation of vWF Thrombocytopenia thrombocytopenia, neuro multimers -> increased platelet Purpura dysfunction (FART'N) aggregation -> small blood clots) plasmaphoresis antibodies (anticardiolipin Anti-phospholipid antibodies, antibodies to autoantibodies against a certain some antibodies may prolong one more of the syndrome/lupus inhibitor B2glycoprotein 1, lupus phospholipid binding proteins phospholipid dependent coagulation test (most Hypercoaguability Disorders syndrome inhibitors) promote thrombosis Prolonged PTT cause a hypercoaguable state. commonly the PTT) most common adult leukemia, slight male predominance, avg age of dx ~67 yo. leukemia accumulates in the bone amrrow and inhibits production of normal, healthy blood cells. discovered incidentally via routine labs or after blood work sidrawn due to nonspecific symptoms: fatigue, weakness, SOB (typically ue to myeloid lineage can be de novo or after progression of MDS or macrocytic anemia), established with at least 20% t(8;21) --> favorable another antecedent bone marrow disease. fever/infections (neutropenia), CD33, CD13, and blasts/promyelocytes/p risk bleeding/bruising (low platelets), MPO, immaturity romonocytes in the inv(16) --> favorable tx in young, fit pts is usually 7+3 7+3 induction chemotherapy is 7 days of aches/pains, weight loss, night established with bone marrow or blood. risk induction chemotherapy +/- stem cytarabine and 3 days of anthracycline: sweats, gum thickeneing. CD34 and CD117. large nucleus with t(15;17) (q22:11-12) -- cell transplant if tolerated idarububicin or daunorubicin. usually requires symptoms typically progress elevated WBC. scant cytoplasm. > medical emergency Bone marrow biopsy often hypomethylating agents several week hopsitalization as it is very toxic. fairly quickly. presents with macrolytic anemia. sometimes have auer but favorable risk required becuase (azacitidine, decitabine) are used causes tumro lysis syndrome, infections, petechiae and ecchymoses due neutropenia. low rods but are typically t(11q23;variable) --> percentage of blasts in in older, frail pts as they are well fatigue, bleeding, need for transfusions, Acute Myeloid Leukemia to thrombocytopenia RBC and platelets. rare monocytic, poor risk blood can be variable tolerated and are out pt tx mucositis, N/V, diarrhea, hair loss myeloid lineage established with CD33, CD13, and MPO, immaturity established with at least 20% ATRA (all trans retinoic acid ) and CD34 and CD117. promeylocytes. large, arsenic trioxide (IV) based therapy. pancytopenia, bruising/bleeding pancytopenia. often bilobed nuclie. t(15;17) (q22;11-12) Start ATRA as soon as dx as it and/or blood clots --> DIC. elevated D- azurophillic (retinoic acid receptor- induces differentiation of Acyte promyelocytic excellent prognosis with high Dimers, low cytoplasmic granules. PML is a tumor promyelocytes and can Acute Myeloid Leukemias leukemia cure rates fibrinogen Auer rods supressor) FISH prevent/reverse the coagulopathy Category Name Presentation Lab findings/ immunophenotype Morphology Biochemistry Genetics Testing Treatment Pathology images Other Notes Other Path Images low risk disease: decrease trransfusion burden and symptoms and improve quality of life if asymptomatic --> observation often with if symptomatic or transfusion chromosomal dependent and anemic--> give abnormalities EPO and lenalidomide (monosomies, if symptomatic or transfusion deletions, trisomies) dependent and NOT anemic --> avg age at dx ~70, male marrow usually hypomethylating agents predominence. incidental at least 1 hypercellular (can be del (5q), del (20q) --> multinucleated red cells (left) and ringed findings or nonspecific symptoms cytopenia normo or hypo). Clonal populations with defective good prognosis higher risk disease: alter natural hx sideroblasts (right) are signs of dysplasia. MDS that depend on blood counts: (macrocytic dysplastic maturation --> cytopenias. may of disease, prevent progression to risk assesment is based on: bone marrow fatigue, weakness, SOB, anemia most differentiation of be primary or secondary (to complex (3 AML, improve overall survival blasts (more = worse) karyotyping (del (5q), bleeding/brusing, common), megakaryocytes, radiation, chemo, or benzene abnormalities, give hypomethylating agents --> del (20q) --> good prognosis. complex (3 fever/infections, aches, weight thrombocytopenia erythroid lineage or exposure). high rate of involvement of dx requires a bone marrow allogenic stem cell transplant if abnormalities, involvement of chromsome 7 --> Myelodysplastic Syndrome Myelodysplastic syndrome loss, night sweats is common myeloid lineage progression to AML chromsome 7 --> poor biopsy eligible poor) avg at is ab 60 yo, often asymptomatic, found from labs. can have fatigue and malaise. splenomegaly (extramedullary hematopoiesis). sx related to splenomegaly: abdominal pain/fullness, early satiety, markedly weight loss. majority of pts dx in increased WBC. hypercellular marrow chronic phase. can progress to increased with overgrowth of accelerated phase (increased granulocytes. maturing granulocytes bone marrow blast 10-19%, increased myeloid (elevated eosinophils worsening cels (especially and basophils). anemia/thrombocytopenia) or neutrophils), megakaryocytes are t(9:22) translocation -- blast crisis (resembles AL with basophilia, usually increased in number > BCR-ABL persistent BCR-ABL, >20% Imatinib (TK inhibitor). consider Chronic Myeloid blasts and or solid focus of blasts Platelets are often erythroids increased TK stem cell transplantation if disease Leukemia outside marrow elevated normal/decreased signaling labs, biopsy, FISH control is attained in blast crisis micromegakaryocytes, small hypolobated pruritis (itchiness after warm shower), splenomegaly, hepatomegaly, erythromelagia. main goal is to reduce risk of can cause arterial or venous thrombosis --> all pts on aspirin thrombosis (acute coronary hypercellular marrow. syndrome is most common elevated increased low risk: aspirin and phlebotomy COD). progression to spent hemoglobin/ megakaryocytes (many phase (marrow fibrosis, hematocrit, low morphologically high risk: aspirin and phlebotomy Polycythemia Vera splenomegaly) or to AML EPO abnormal) JAK2 mutation and hydroxurea can be asymptomatic or present similarily to PV (pruritis in ET is main goal is to reduce risk of rare). rarely progresses to spent elevated platelets. thrombosis --> all pts on aspirin phase or AML. extramedullary normo/mildly hematopoiesis/organomegaly in hypercellular increased and JAK 2 (30-50%) or low risk: aspirin and observation Essential 50% of cases. elevated platelets marrow. lack of atypically large MPL or CALR Thrombocythemia increases risk of thrombosis true fibrosis platelets mutations high risk: aspirin and hydroxurea early phase may resemble PV or ET. progressive replacement of marrow space by fibrosis without preceding MPD. premature forms of cells are released some have systemic symptoms into blood while bone of fatigue, night sweats, marrow spaces ossify early stages --> symptom control unexplained weight loss, early leukocytosis. --> worsening JAK 2 (30-50%) or Myeloproliferative satiety, fevers/chills. massive anemia/thrombocyt cytopenias. dacrocytes MPL or CALR later stages --> allogenic stem cell Neoplasms Primary Myelofibrosis hepatosplenomegaly openia (tear drop cells) mutations transplant Hyperdiploidy --> establish lymphoid good lineage with TdT hypodiploidy --> poor and CD34. in B-ALL, targeted therapies can be used in Usually leukemic; lymphoblasts established as B- Balanced relapse: fill the marrow. presents with ALL with CD10 Large nuclei, scant translocations: multi-agent chemo Blinatumomab (no chemo, 2 sites) or B cell Acute fever, bone pain, weakness, and and CD19 as well cytoplasm. high mitotic t(9;22) --> poor Inotuzumab Ozogamacin )includes chemo, 1 Lymphoblastic Leukemia fatigue as TdT/CD34 rate t(12;21) --> good in B-ALL, add TK inhibitor site) (imatinib) if philly chromosome positive allogeneic stem cell transplantation performed in high establish lymphoid risk disease lineage with TdT Usually lymphomatous; and CD34. CNS prophylaxis given to all pts as mediastinal mass. presents with established as t- Large nuclei, scant risk of CNS disease is high Acute Lymphoblastic T cell acute lymphoblastic fever, bone pain, weakness, and ALL with CD5, cytoplasm. high mitotic (intrathecal chemotx - Leukemias leukemia fatigue CD7,and CD3 rate NOTCH1 methotrexorate and cytarabine) Nodular sclerosis: fibrous bands that divide mass into nodule, most common. low EBV association Mixed cellularity: assoc with ABVD: IV day 1 and day 15 every 28 days immunodeficiency, abundance Adriamycin (Adverse effects: Cardiotoxicity, cell mix in background risk of secondary cancer, alopecia. Need Originates in germinal center and (eosinophils, plasma cells, screening echocardiogram prior to starting marginal zone B cells. Bimodal lymphocytes, macrophages). Limited stage: 3 cycles of combo treatment) age of occurence (15-35 and >55 moderate EBV association ABVD +/- local radiation tx (85% Bleomycin (Adverse effects: Pulmonary yo) with more males. lymphocyte rich: best prognosis, are disease free ten years later) toxicity. Omit after 2 cycles if patient Lymphadenopathy. B symptoms background=mostly small responding or omit initially and combine AVD + (fever >100.4 F, drenching nigth Reed sternberg cells lymphocytes). low EBV Advanced Stage: 6 cycles of AVD Brentuximab. Need screening pulmonary sweats, unintentional loss of (big prominent nuclei association IHC, PET-CT, CBC, CMP, based chemotx (70% disease free function tests (PFTs) prior to starting >10% of one's body weight), and nucleolus, lots of lymphocyte depleted: worst LDH,Uric acid, HIV 10 years later) treatment) pruritis. alcohol-induced pain. cytoplasm), and prognosis. associated with serologies, Hepatitis Vinblastine (Adverse effects: Neuropathy) continuous, orderly spread of elevated LDH, reactive infiltrate immunodeficiency (often HIV). serologies. reactive infiltarte Relapsed disease: salvage chemo Dacarbazine (Adverse effects: Cytopenias, disease (lymph node -> spleen - CD30+ and CD15+ (lymphocytes, plasma High EBV association limits utility of flow for and autologous stem cell nausea) Mature Cell Lymphomas Hodkin Lymphoma > liver -> bone marrow) and CD20- cells, eosinophils) testing. transplant, brentuximab, anti-PDl1 Category Name Presentation Lab findings/ immunophenotype Morphology Biochemistry Genetics Testing Treatment Pathology images Other Notes Other Path Images Limited stage: 3 cycles of RCHOP Advanced stage: 6 cycles of RCHOP + raadiation therapy for IHC, PET-CT, CBC, CMP, bulky disease R-CHOP → IV every 21 days LDH,Uric acid, HIV Rituximab – anti-CD20 monoclonal antibody Originates in germinal center serologies, Hepatitis if HIV + or have adrenal, testes, or (Adverse effects: Infusion reactions) (better prognosis) and marginal serologies. Flow kidney imvolvement: intrathecal Cyclophosphamide (Adverse effects: zone (worse prognosis) B cells. stain for Diffuse pattern of cytommetry may be false chemotx needed - CNS disease cytopenias) Pts present with bulky tumor CD19+, CD20+, growth. large cells, negative because the cells prophylaxis Doxorubicin (Adverse effects: cardiotoxicity, mass, extranodal disease, CD10+, Bcl2- if usually pleomorphic. die quickly. alopecia (loss of hair). Need screening presence of B symptoms. Some germinal and may resemble Hodkin Virally associated subtypes: C-MYC translocation, Immunopheotype helps If relapse: salvage chemotx and echocardiogram) pts may have indolent B cell CD19+, CD20+, lymphoma or non- immunodeficicny associated BCL6 translocation, distringuish between autologous stem cell transplant. Vincristine (Adverse effects: neuropathy) Diffuse large B- cell lymphoma that transforms into Bcl2 + if marginal lymphomatous large B-cell lymphomas (HIV, BCL2 translocation --> germinal center and non- CAR T cell therapy (for second Prednisone - oral steroid pills x 5 days lymphoma diffuse large B cell lymphoma zone. malignancy post-transplant, or EBV driven) t(14:18) germinal center BLBCL relape and beyond) medium sized cells Originates in germinal center B with little cytoplasm cells. presents as a rapidly (monotonous), high growing mass. doubling time of rate of apoptosis Histologically identical subtypes: tumor of less than 24 hrs. high (macrophages with endemic (african), practically all c-MYC translocation incidence of tumor lysis dead cells -> starry EBV on chromosome 8 IHC, PET-CT, CBC, CMP, syndrome. rapidly fatal. stain for CD19+, sky), extremely high sporadic - minority are EBV t(8;14) - IgH locus LDH,Uric acid, HIV B cell Lymphomas (high increased incidence in CD20+, CD10+, rate of mitosis ( Ki67 assoc t(2;8) - IgK serologies, Hepatitis grade/ aggressive) Burkitt Lymphoma immunocopromised indis Bcl2- ~100%), HIV - minority are EBV assoc t(8;22) - IgY serologies. infusional chemotx involvement of nodes, liver, spleen, marrow, and blood. small lymphocytes with originates in naive B cells (worse mature B cell pathognomic prognosis) and memory cells markers and proliferation centers chromosomal (better prognosis). presents in surface Ig -> (psuedofollicles). deletions and older adults (~70 yo). can have a CD19+, CD20+ but smudge cells are not duplications are richter transformation to diffuse dim, CD5, CD23. pathognomic for CLL. common (tend to have treat only when symptomatic (e.g. Chronic lymohocytic large B cell lymphoma. pt can be elevated WBC with presence of somatic trisomies and when pts acquire new cytopenias). leukemia/small asymptomatic (found on routine lymphocyte hypermutation is deletions, not targeted therapy: ibrutinib(bruton's lymphocytic leukemia lab work) predominance favorable prognosis translocations) tyrosine kinase inhibitor) good performance status:in pt Originates in mantle B cells. chemo regimen with cytrabine. if Morphologically low grade, good response, have autologus clinically aggressive. presents in stem cell transplant. older adults (~63 yo, male predominace). presents with poor performance status: outpt lymphadenopathy, extranodal chemo with bendamustine and site involvement (GI tract and rituximab then rituximab maintence waldeyer ring: ring of lymphoid lymphoma cells are balanced translocation for 2 yrs tissues around the tonsils). most CD19+, CD20+, small because this is a between cyclin D1 and cyclin D1 is an oncogene that inactivates the pts have marrow and peripheral CD5+, Cyclin D1+, low grade malignancy. Ig heavy chain: t (11; CAR-T cell therapyrecently RB tumor suppressor. Inactivation allows for Mantle Cell Lymphoma blood involvement at dx CD23- very monotonous. 14) approved cell proliferation low stage, isolated site: radiation alone low or advanced stage with b symptoms/cytopenias/bulky Originates in germinal center B disease/lymphoma related organ cells. Presents in older adults. dysfunction: bendamustine and Involves the lymph nodes, rituximab spleen, liver, and marrow. may lots of neoplastic transform to more aggressive follicles crowding out advanced stage, asymptomatic: lymphoma. riskof transformation normal nodal observation to higher grade disease (usually architecture. BLBCL). Majority of pts have composed of a mix of relapsed disease: targeted oral or advanced stage disease at dx. small and large cells IV therapy usually involves lymph nodes, (the more large cells, spleen, marrow. median survival the worse the Translocation between transformed disease: more tx- is 8-10 yrs without tx. incurable CD19+, CD20+, prognosis). somatic Bcl2 and Ig heavy refractory than de-novo DLBCL Follicular Lymphoma but manageable CD10+, Bcl2+ hypermutation. chain t(14;18) MALT lymphoma with chronic inflmattion: tx underlying inflammation MALT lymphoma without chronic originates in marginal zone b inflammation, Non-MALT and cells. mucosal associated moderate cytoplasm asymptomatic: observation lymphoid tissue (MALToma). (fairly normal looking). often associated withchronic post germinal center MALT lymphoma without chronic inflammation (H pylori). May marginal zone cells on inflammation, Non-MALT and regress if cause of inflmmation is no specific cusp of becoming translocations: t(11; symptomatic: rituximab x 4-8 Marginal zone lymphoma tx. markers plasma cells 18) - AP12-MALT1 weeks Single agent cladribine (adenosine analog, insertion blocks S phase replication) originates in memory cells. Common in elderly males. often Rituximab involves spleen, marrow, and B cell lymphomas (low grade/ blood. associated with marrow TRAP stain if BRAF v600e mutation: BRAF indolent) Hairy cell leukemia fibrosis. positive hairy looking cells inhibitor vemurafenib elevated CBC, Globulin gap monoclonal (calculated via CMP via paraprotein blood subtracting total protein - (M protein) albumin), Ca2+ studies, Asymptomatic, preneoplastic measuring 3 g/dL (coin stack) immunofixation no tx Category Name Presentation Lab findings/ immunophenotype Morphology Biochemistry Genetics Testing Treatment Pathology images Other Notes Other Path Images CBC, Globulin gap Typically bony lesions but can be (calculated via CMP via found anywhere in the body ( subtracting total protein - bony -> medullary, soft tissue -> Will show clonality albumin), Ca2+ studies, extramedullary). will often have for either kappa or serum kappa to lambda concurrent monoclonal lambda. ratio, levels of Ig heavy gammopathy (secreted associated with rouleaux formation chains, serum protein immunoglobulin detected in specific heavy (coin stack). sheets of electrophoresis, and radiation of localized, chemotx if Plasmacytomas serum M protein) chain (mostly IgG) clonal plasma cells immunofixation systemic Majority of pts present with signs/sx related to infiltration of for fit, transplant eligible pts: triplet plasma cells into bone or other chemotx x 3-4 months, if remission organs. anemia, bone pain, CBC, Globulin gap achieved, autologous stem cell acute renal dysfunction, fatigue, (calculated via CMP via transplant then maintenace hypercalcemia. can becoem a Charcaterized by either subtracting total protein - therapy medical emergency from cord >60% clonal plasmacytomas or albumin), Ca2+ studies, compression (impingement of plasma cells or 10- increased plasma serum kappa to lambda for fit, transplant ineligible pts: spinal cord due to extramedullary 60% clonal plasma clonal cells in marrow ratio, levels of Ig heavy triplet chemotx for 8-12 months SLiM- CRAB plasmacytoma) which can cause cells and one of with normal and chains, serum protein then maintenance therapy Sixty% bone marrow plasmacytosis severe back pain, weakness of the LiM CRAB abnormal plasma cell electrophoresis and Light chains involved: uninvolved >100 lower extremities, and criteria. globulin forms and multiple immunofixation, whole body for frail patients, doublet tx MRI >1 focal lesiom bladder/bowel incontinence or gap. normocytic molecular defects and CT scan (skeletal survey) to (lenalidomide and dexamethasone C (hyper) calcemia exophthalmos. median age at dx anemia. elevated cytogenetic check for lytic lesions, Renal failure is 65-74 yo with a slight male calcium and abnormalities biopsy of plasmacytomas signs of cord compression --> Anemia Plasma cell neoplasms Multiple Myeloma predominance creatine (rouleaux formation) and bone marrow emergent radiation therapy Bon lytic lesions Pathologic misfolding of proteins into beta pleated sheets. the Impaired kidney function, but can misfolded proteins are deposited also have hematological issues between cells in various tissues like anemia, thrombocytopenia, and organs in a wide variety of neuropathy, musculoskeletal Congo red stain under clinical settings. Light chains Secodnary amylpidosis is caused by fibril Immunoglobulin issues, cardiac issues, GI poalrized light - apple- produced by myeloma cells are protein serum amyloid A (AA) and is seen in Abnormalities Primary Amyloidosis (macroglossia, hepatomegaly) green birefringence often a cause chronic inflammatory conditions chemotx, if remission with chemotx, follow with consolifdative Lymphadenopathy, tumor cells with HTLV-1 associated (endemic to autologous stem cell transplant. if Adult T cell hepatosplenomegaly, skin cloverleaf pacific islands, carribean relapsed disease, use allogenic leukemia/lymphoma lesions, blood multilobulated nuclei islands, parts of africa, korea) stem cell transplant Destructive massof chemotx, if remission with nasopharynx. skin, testes can chemotx, follow with consolifdative also be involved. invades autologous stem cell transplant. if Viral Association (T/NK Extranodal NK/T cell vessels and causes ischemic relapsed disease, use allogenic lymphomas) lymphoma necrosis strong association with EBV stem cell transplant large anaplastic cells clustered around ALK-1 constitutively vessels and activated by chemotx, if remission with lymphatics./ may mimic translocation of gene chemotx, follow with consolifdative Visceral Involvement, carcinoma. hallmark on chromosome 2. autologous stem cell transplant. if specific translocation (T/NK Anaplastic large cell cells that looks like ALK- have a worse relapsed disease, use allogenic lympomas) lymphoma Often involves the soft tissue horseshoes prognosis stem cell transplant Infiltration of the skin, with late phase node and marrow involvement. Sexary syndrome Malignant T helper involves the skin, but as cells inffiltrate the skin, Mycosis generalized process without loss of CD7 which forming plaques and Skin Involvement (T/NK Fungoides/Sezary discrete tumors and is asociated should be present tumors. cerebriform T tx may involve UV light therapy or lymphomas) syndrome with a leukemic presentation on all T cells cells. chemotx Heterogenous collection of several T cell lymphocytes that dont fit into other categories, effacement of lymph node architectyre, pleomorphic malignant cells or can resemble chemotx, if remission with reactive cells. Mature T chemotx, follow with consolifdative cells often with 1+ autologous stem cell transplant. if Visceral Involvement, NOS Peripheral T cell immunophenotypic relapsed disease, use allogenic (T/NK lymphomas) lymphoma, NOS abnormality stem cell transplant If multifocal, masses in bone, liver, spleen, skin, lung, soft tissue.and is very aggresive dendritic cell If unisystem, usually masses in neoplasm. grooved bone. cells "coffee beans" Langerhans Cell Langerhans Cell If pulmoanry, usually in adult S-100+ and and birbeck granules Histiocytosis Histiocytosis smokers and may be reactive CD1a+ (EM)