Akutt leukemi - Amboss

Question 1

Hvordan er epidemiologien til akutt lymfatisk leukemi (ALL)?

Answer 1

Question 2

Hvordan er epidemiologien til akutt myeloid leukemi (AML)?

Answer 2

Question 3

Hvilke etiologi har ALL?

Answer 3

More commonly associated with AML than ALL.

Question 4

Hvilken etiologi har AML?

Answer 4

Some hematopoietic disorders can progress to AML, although this process may take months or even years.

Question 5

Hvordan klassifiserers ALL ved bruk av FAB?

Answer 5

Question 6

Hvordan klassifiserer ALL jmf. WHO?

Answer 6

Precursor lymphoblastic leukemia encompasses the historical FAB classification L1 and L2 subtypes. L3 (Burkitt lymphoma) is now classified as a mature B-cell lymphoma.

Question 7

Hvordan klassifiserer man ALL basert på immunofenotyping?

Answer 7

Burkitt lymphoma with secondary marrow or peripheral blood involvement.

Question 8

Hva viser bildet?

Answer 8

B-cell acute lymphoblastic leukemia: Photomicrographs of a peripheral blood smear (H&E stain; very high magnification); The large basophilic cell in the center of each photomicrograph is a lymphoblast, identifiable by its large spherical nucleus surrounded by a thin rim of cytoplasm (high nucleocytoplasmic ratio). The nucleus of the lymphoblasts is often indented (green arrows) and shows fine loose (noncondensed) chromatin. Lymphoblasts differ from lymphocytes, which are smaller in size with smaller nuclei, condensed chromatin, and more cytoplasm. The presence of numerous lymphoblasts in a peripheral blood smear is indicative of lymphoblastic leukemia. The diagnosis of B-cell ALL was confirmed on immunophenotyping. Note: Basophilic remnants (Howell-Jolly bodies) are visible within the erythrocytes in the last photomicrograph, indicating (in this case) increased hematopoiesis.

Question 9

Hva viser bildet?

Answer 9

T-cell acute lymphoblastic leukemia: Photomicrographs of a peripheral blood smear (H&E stain; very high magnification); The basophilic cell clusters in the center of each photomicrograph are mainly composed of lymphoblasts, identifiable by their large size, large spherical nucleus that is surrounded by a thin rim of cytoplasm (high nucleocytoplasmic ratio). The nucleus of the lymphoblasts is often indented and shows fine loose (noncondensed) chromatin. Lymphoblasts differ from lymphocytes, which are smaller in size with smaller nuclei, condensed chromatin, and more cytoplasm. The presence of numerous lymphoblasts in a peripheral blood smear is indicative of lymphoblastic leukemia. The diagnosis of T-cell ALL was confirmed on immunophenotyping. Note: Basophilic remnants (Howell-Jolly bodies) are visible within some of the erythrocytes, indicating (in this case) increased hematopoiesis.

Question 10

Hvordan klassifiserer AML jmf. FAB?

Answer 10

The French-American-British (FAB) classification distinguishes between eight subtypes of AML, according to the histopathological appearance of the cells.

Question 11

Hvordan klassifiserer AML jmf. WHO?

Answer 11

Question 12

Hva viser bildet?

Answer 12

Acute monocytic leukemia: Photomicrographs of a bone marrow smear (H&E stain; very high magnification); The large basophilic cell visible in each photomicrograph is a monocyte, identifiable by its large size, folded or lobed nucleus with fine chromatin and prominent nucleoli (green overlay), and abundant granular cytoplasm with cytoplasmic vacuoles. Numerous monocytes on bone marrow biopsy is characteristically seen in acute monocytic leukemia. The diagnosis is confirmed on immunophenotyping.

Question 13

Hva viser bildet?

Answer 13

Acute promyelocytic leukemia; Photomicrographs of a peripheral blood smear (H&E stain; very high magnification): The large basophilic cells are promyelocytes, identifiable by their eccentric nuclei and abundant basophilic cytoplasm containing numerous Auer rods (rod-like intracytoplasmic inclusion bodies). This type of hypergranular leukemic promyelocyte is often still referred to as a “faggot cell” for the resemblance of accumulation of Auer rods to a “bundle of sticks,” although the term is typically avoided today due to its derogatory associations. The presence of promyelocytes containing Auer rods in a peripheral blood smear is characteristic of acute promyelocytic leukemia. Note: Basophilic remnants (Howell-Jolly bodies) are visible within most of the erythrocytes, indicating (in this case) increased hematopoiesis.

Question 14

Fyll inn figuren

Answer 14

Hematopoiesis; The stages of hematopoiesis, a process by which pluripotent hematopoietic stem cells in the bone marrow differentiate into two main types of precursor cells: myeloid and lymphoid. Myeloid precursor cells differentiate into erythrocytes, leukocytes, and thrombocytes. Lymphoid precursor cells differentiate into natural killer cells (NK cells) and B and T lymphocytes. Dendritic cells, which are antigen-presenting cells, develop from both myeloid and lymphoid precursors.

Question 15

Hvordan er patofysiologien ved akutt leukemi?

Answer 15

Mutations result in activation or inactivation of genes (e.g., Philadelphia chromosome, t(15;17)) that affect the proliferation and maturation of bone marrow cells.

Question 16

Hva er klinikken ved akutt leukemi avhengig av?

Answer 16

Clinical features are either related to bone marrow failure, infiltration of organs by leukemic cells, or a combination of both.

Question 17

Hvilke generelle kliniske tegn forekommer ved akutt leukemi?

Answer 17

Some patients report having nonspecific symptoms (fatigue) for a few months prior to presentation. Hepatosplenomegaly is more common in ALL than AML, occurring in > 60% of individuals with ALL and approx. 10% of individuals with AML. In AML, it may suggest the presence of a previous myeloproliferative disorder.

Question 18

Hvilken klinikk forekommer ved ALL?

Answer 18

Fever and lymphadenopathy are rare in AML, but can be common first signs in ALL!

Remember metastasis for ALL by thinking of the following: ALL metaStaSizeS to the CNS and teSteS.

Note that fever as a constitutional symptom in the absence of infection is typical for ALL, but not AML.

Question 19

Hvilken klinikk forekommer ved AML?

Answer 19

Caused by myeloblasts infiltrating the mucosa.

Question 20

Hvordan skal feber hos en pasient med akutt leukemi tolkes?

Answer 20

Fever in a patient with acute leukemia must always be treated as a sign of infection until proven otherwise!

Question 21

Hva viser bildet?

Answer 21

Leukemia cutis; Multiple erythematous papules and nodules are seen on the face and neck of this patient as a result of infiltrating malignant neoplastic cells accumulating in the dermis.

Question 22

Hvordan tilnærmer man seg en pas. med mistenkt akutt leukemi?

Answer 22

Question 23

Hva kan man si om funnene på evt. blodprøver ved akutt leukemi?

Answer 23

Findings on initial laboratory studies are usually nonspecific but may help to identify potentially life-threatening acute complications.

Question 24

Hvilke funn forekommer ved akutt leukemi av hhv.:
- Leukocytter
- Trombocytter
- Hemoglobin
- Blodutstryk

Answer 24

Although the WBC count may be normal or even elevated in patients with acute leukemia, the WBCs will be immature and nonfunctional. In some patients, levels of only certain types of WBC will be elevated, e.g., hypereosinophilia may be present prior to an ALL diagnosis. Even in mild thrombocytopenia, several different platelet abnormalities may be present (e.g., abnormal size and shape on smear), which may affect their function. This can result in impaired aggregation and adherence.

Question 25

Hva kan man se på lever- og nyreprøvene ved akutt leukemi?

Answer 25

Question 26

Hvilke funn kan forekomme ved utvidet metabolsk panel ved akutt leukemi?

Answer 26

Question 27

Hva kan koagulasjonsprøver vise ved akutt leukemi?

Answer 27

Question 28

Hvilke histopatologiske funn er typiske for ALL?

Answer 28

Cell morphology can confirm the diagnosis of acute leukemia, but in most cases, it is necessary to complete immunophenotype and genetic studies before selecting a treatment.

Question 29

Hva er histopatologiske funn ved AML?

Answer 29

Cell morphology can confirm the diagnosis of acute leukemia, but in most cases, it is necessary to complete immunophenotype and genetic studies before selecting a treatment.

Question 30

Hva er "leukemisk hiatus"?

Answer 30

Question 31

Hva viser bildet?

Answer 31

Question 32

Hva viser bildet?

Answer 32

Question 33

Hva viser bildet?

Answer 33

Question 34

Hva viser dette bildet?

Answer 34

Question 35

Hva viser dette bildet?

Answer 35

Question 36

Hva viser bildet?

Answer 36

Question 37

Hva viser pilen til?

Answer 37

Question 38

Hva viser pilene til?

Answer 38

Question 39

Hva er "Philadelphia kromosomet"?

Answer 39

Question 40

Hvilke forskjeller er det mellom immunofenotypingen til ALL og AML?

Answer 40

Question 41

Hvilke forskjeller er det mellom ALL og AML når det kommer til genetiske studier?

Answer 41

Question 42

Hvilke screeningmuligheter har man for pas. med akutt leukemi?

Answer 42

Question 43

Hvilken type behandling gir man pas. med akutt leukemi?

Answer 43

Question 44

Hvor lenge bør kjemoterapien vare ved akutt leukemi?

Answer 44

Regimens vary depending on the subtype of leukemia, the age of the patient, and immunophenotype and genetic study results.

Question 45

Hvilke medikamentelle kjemopreparater brukes ved ALL?

Answer 45

A chemotherapy regimen commonly used to treat *ALL* is hyper-**CVAD**: **C**yclophosphamide, **V**incristine, daunorubicin (or **A**driamycin), and **D**examethasone.

Question 46

Hvilke medikamentelle kjemopreparater brukes ved AML?

Answer 46

Question 47

Hvilke typer medikamentelle kjemopreparater brukes ved APL?

Answer 47

If APL is suspected, start treatment early with a differentiation agent (e.g., ATRA) without waiting for immunotype or genetic confirmation. Treatment may be adjusted later depending on the results. In APL, the **t(15;17) translocation** and subsequent formation of the *PML-RARA* fusion gene can inhibit myeloblast differentiation under physiological levels of retinoic acid. **High doses of ATRA (a vitamin A derivative)** may induce myeloblast differentiation and **promote remission**.

Question 48

Hvordan vil man behandle CNS infiltrasjon ved akutt leukemi?

Answer 48

Question 49

Hvilke type behandling er målrettet ved akutt leukemi?

Answer 49

Question 50

Hvilken annen spesialbehandling er mulig ved akutt leukemi?

Answer 50

Autologous or allogeneic stem cell transplantation: Indications include patients with poor prognostic factors (e.g., unfavorable cytogenetics) and those who do not achieve remission with chemotherapy.

Question 51

Hvilke onkologiske nødsituasjoner kan oppstå ved akutt leukemi?

Answer 51

Question 52

Hvilke behandlingsrelaterte nødsituasjoner kan oppstå ved akutt leukemi?

Answer 52

Question 53

Hva kan en sekundær hyperuremi føre til ved akutt leukemi?

Answer 53

Question 54

Hvordan er 5-årsoverlevelsen til ALL og AML?

Answer 54

Question 55

Hvilke prognostiske faktorer er ugunstige ved ALL/AML?

Answer 55

Question 56

Hvilke gunstige prognostiske faktorer finner man ved ALL/AML?

Answer 56

To remember that translocation **t(12;21)** commonly manifests with **pediatric B-ALL** and usually has a **favorable outcome**, think: **“Kids flip back to health!”** (the **number 12** is **21 flipped around**).