{"id":10470,"date":"2022-02-06T22:24:56","date_gmt":"2022-02-06T20:24:56","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/summary-of-hematological-malignancies\/"},"modified":"2022-02-07T00:07:13","modified_gmt":"2022-02-06T22:07:13","slug":"summary-of-hematological-malignancies","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/summary-of-hematological-malignancies\/","title":{"rendered":"Summary of hematological malignancies"},"content":{"rendered":"\n

Acute leukemias<\/h4>\n<\/span>\n\n\n
<\/th>AML<\/th>ALL<\/th><\/tr><\/thead>
Genetics<\/strong><\/td>Proliferative:<\/strong> FLT3, JAK-2, C-KIT, ABL1
Inhibition of differentiation: <\/strong>RARA, NPM1
Epigenetic modulators:<\/strong> DNMT3A<\/td>		NOTCH1, WT1, EZH2
t(12;21), t(9;22) <\/td><\/tr>
Epidemiology<\/strong><\/td>Older adults<\/td>Children and older adults<\/td><\/tr>
Staging\/classification<\/strong><\/td>FAB<\/strong> (M0-M7)
WHO <\/strong>(I-VI)<\/td>		FAB<\/strong> (L1-L3)
WHO <\/strong>(13 groups)<\/td><\/tr>
Additional information<\/strong><\/td>Acute promyelocytic leukemia<\/strong> (APL) is a type of AML, WHO class I with t(15;17)
RA receptors are disrupted, promyelocytes accumulate with Auer rods (MPO aggregation; risk for DIC)<\/td>		t(12;21) has a\u00a0good prognosis, mostly seen in children

t(9;22) has a bad prognosis, mostly seen in adults (Philadelphia chromosome; BCR-ABL fusion)<\/td><\/tr>
Treatment<\/strong><\/td>Induction<\/strong>: Cytarabine + anthracyclin (7+3)
Postinduction<\/strong>: Several doses of high-dose cytarabine
In FLT3 mutations<\/strong>:\u00a0midostaurin
Anti-CD33 mAbs<\/strong>:\u00a0gemtuzumab ozogamicin
BMT<\/strong>
ATRA <\/strong>(in APL, maturates promyelocytes)<\/td>		Induction<\/strong>: Vincristine + corticosteroids +- daunorubicin (4-6 weeks)
Consolidation<\/strong>: Cyclophosphamide\/cytarabine\/6-MP\/daunorubicin (6-9 months)
Maintenance<\/strong>: 6-MP\/MTX +-corticosteroids (3 years)
BMT<\/strong>
TK inhibitors<\/strong>: imatinib (in BCR-ABL1)<\/td><\/tr><\/tbody><\/table><\/figure>\n\n\n\n
  • Tx for AML
    • CytAn<\/li>
    • Cyt<\/li>
    • Mid<\/li>
    • GO<\/li><\/ul><\/li>
    • Tx for ALL
      • VinCoDa<\/li>
      • CycCytMPDa<\/li>
      • MPMetCo<\/li>
      • Ima<\/li><\/ul><\/li><\/ul>\n\n\n<\/span>\n

        Chronic leukemias\/lymphomas<\/h4>\n<\/span>\n\n\n
        <\/th>CML<\/th>CLL<\/th><\/tr><\/thead>
        Genetics<\/strong><\/td>t(9;22)\u00a0BCR-ABL1<\/td>–<\/td><\/tr>
        Epidemiology<\/strong><\/td>Older adults<\/td>Older adults
        The most common type of leukemia<\/td><\/tr>
        Staging\/classification<\/strong><\/td>Sokal<\/strong>,\u00a0Hasford<\/strong>, and\u00a0EUTOS<\/strong> systems based on:
        Age of the patient
        The proportion of blast cells
        Spleen size
        Platelet count<\/td>        		Rai<\/strong> (0-4) and Binet<\/strong> (A-C) systems based on lymphocytosis, lymphatic involvement, anemia, and thrombocytopenia.<\/td><\/tr>
        Additional information<\/strong><\/td>Phases<\/strong>: chronic (<10% immature cells), accelerated (10-20%), blast (>20%)

        GAPA <\/strong>or LAP (leukocyte alkaline phosphatase) score reflects the intracellular alkaline phosphatase activity of leukocytes; to measure it, 100 leukocytes are examined and scored 0-4; in CML, the GAPA is low

        Response to TK inhibitors is the most important prognostic factor<\/td>        		Blood smear shows small lymphocytes with smudge or Gumprecht\u00a0shadow<\/em> cells; surface markers include CD5, CD38, ZAP70

        Can progress into B-cell prolymphocytic leukemia and DLBCL

        CLL and SLL (small lymphocytic lymphoma) are identical, except in CLL the malignant cells are found mostly in the blood and bone marrow; therefore, they are often grouped together as CLL\/SLL under non-Hodgkin lymphomas.

        IgHV (immunoglobulin heavy chain variable) mutations are said to be equivalent to somatic hypermutation, serving as a\u00a0positive prognostic factor<\/strong>.
        They are considered mutated If the difference of the IgHV gene sequence is >2% from the germline sequence.

        Hypogammaglobulinemia and monoclonality lead to immune suppression and autoimmunity.

        Richter\u2019s syndrome is a transformation to a more aggressive disease.<\/td><\/tr>
        Treatment<\/strong><\/td>TK-inhibitors<\/strong>: imatinib, nilotinib, dasatinib, ponatinib is used in resistance
        Chemotherapy <\/strong>(hydroxycarbamide)
        BMT<\/strong><\/td>        		FCR<\/strong>: fludarabine + cyclophosphamide + rituximab
        BTKi<\/strong>: Ibrutinib
        VO<\/strong>: Venotoclax + obinutuzumab
        BR<\/strong>: bendamustine + rituximab<\/td><\/tr><\/tbody><\/table><\/figure>\n\n\n\n
        • Tx for CML: ImaNiloDasaPon<\/li>
        • Tx for CLL:
          • FCR<\/li>
          • Ibru<\/li>
          • VO<\/li>
          • BR<\/li><\/ul><\/li><\/ul>\n\n\n<\/span>\n

            Additional information<\/h4>\n<\/span>\n\n\n
            • FAB = French-American-British<\/li>
            • Cell markers:
              • Myeloid: CD13, CD33, and MPO positive; TdT negative<\/li>
              • Lymphoblasts: TdT positive<\/li>
              • B-cell-specific markers: CD19, CD20<\/li>
              • T-cell-specific markers: CD2, CD3, CD7<\/li>
              • NK-cell-specific markers: CD56<\/li><\/ul><\/li>
              • Tyrosine-kinase inhibitors work by binding to the BCR-ABL1 tyrosine-kinase instead of ATP<\/li>
              • Fludarabine = purine analog<\/li>
              • Cytarabine = pyrimidine analog<\/li>
              • Anthracyclin = antibiotic<\/li>
              • Daunorubicin = antibiotic<\/li>
              • Cyclophosphamide = alkylating agent<\/li>
              • Venotoclax = BCL-2 inhibitior<\/li>
              • Obinutuzumab = anti-CD20<\/li>
              • Bendamustine = alkylating agent<\/li><\/ul>\n<\/span>

                Acute leukemias<\/h4>

                Chronic leukemias\/lymphomas<\/h4>

                Additional information<\/h4><\/div>","protected":false},"excerpt":{"rendered":"

                Acute leukemias AML ALL Genetics Proliferative: FLT3, JAK-2, C-KIT, ABL1Inhibition of differentiation: RARA, NPM1Epigenetic modulators: DNMT3A NOTCH1, WT1, EZH2t(12;21), t(9;22) Epidemiology Older adults Children and older adults Staging\/classification FAB (M0-M7)WHO (I-VI) FAB (L1-L3)WHO (13 groups) Additional information Acute promyelocytic leukemia (APL) is a type of AML, WHO class I with t(15;17) RA receptors are disrupted, […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5331,"menu_order":6,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-10470","page","type-page","status-publish","hentry"],"yoast_head":"\nSummary of hematological malignancies – Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" 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