Myeloproliferative Disorders, Lec 10
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| Meyeloid Proiferative disorders imply that...? | Myeloid cell line is involved and is about making more cells.
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| What is Chronic myeloid leukemia is characterized by? | Extreme elevation of myeloid cells in blood, and increased myeloid cell in BM. Insidious onset.
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| What are 3 phases of Chronic Myeloid Leukemia? | 1) chronic phase
2) Accelerated phases
3) Blast crisis
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| What may Chronic Myeloid Leukemia transform into? | ALL (acute lymphocytic leukemia) or AML (acute myeloid leukemia)
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| What is they typical phylogenetic change in chronic myeloid leukemia? | Philadelphia chromosome = transition of Tyrosine base from pos 9 to pos 22. Makes bcr-abl product. Tyrosine kinase is always on, so it can't control cell maintenance.
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| What do additional mutations of Chronic myeloid leukemia lead to? | Blast crisis phase
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| What is the presentation of Chronic Myeloid leukemia? | Unexplained fever, weakness, bleeding (fewer plts are produced). Physical exam shows: pallor (due to anemia), tenderness over lower sternum expansion of BM), splenomegaly (spleen pulls out lots of abnormals), no lymphadenopathy (Lymphoids are NOT swollen)
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| Why is they no lymphadenopathy in chronic myeloid leukemia? | Bc it's a myeloid disease, not a lymphoid disease so the lymphoid is not gonna get bigger.
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| Why can you see Petechia and ecchymoses in Chronic Lymphoid Leukemia? | bc there are quantitative and qualitative defects in platelets
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| What are lab findings for Chronic Myeloid Leukemia? | -High WBC count (>100x10^9) w/left shift (more immature cells)
-Thrombocytosis (sometimes)
-Abnormal Plt function often
-Norm/Norm anemia (leukocytosis high=anemia high, low Hgb)
-Increased basophils (20%)
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| T or F: Hgb count is INVERSELY proportional to WBC count in Chronic Myeloid Leukemia. | True
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| Predominant cells types in peripheral blood are in Chronic Myeloid Leukemia is? | Metamyleloytes, banded netrophils (immature cell, promyelocytes. Lots of Myeloid precursors
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| What will you find in the BM of Chronic Myeloid Leukemia? | -90-100% cellular (as opposed to normal 50%)
-<20% blasts (>20% is acute leukemia!!)
-Increased Normoblasts and Megakaryoblasts
-M:E ratio is 10:1 or 50:1 (as opposed to normal 2:1)
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| What is the difference between Chronic and Acute leukemia? | Chronic has <20% blasts and Acute has >20% blasts in the blood
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| What is the progression of Chronic Myeloid Leukemia? | 30-40 mo. after diagnosis. Increased splenomegaly, rising Luekocytosis (faster cell cycle, bc they are released from cell cycle contorl), Prominent Basophilia
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| What is blast crisis? | After accumulation due to progression, there is >20% blasts in BM or PB. NOT called AcuteML bc there are preexisting CML diagnosis (an elaboration of the previous condition).
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| How do you treat Blast crisis? | Gleevec, a drug based on the protein morphology of that which causes Chronic Myeloid Leukemia.
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| What is the difference between Leukemoid Reaction from CML? | CML (cells develop abnormally)has HIGH Eos/Basos, Low LAP, Ph chromosome (+), INC plts, and anemia with nRBCs. Leukamoid reaction (see signs of Oxidation of WBCs)has NORM Eos/Basos, High LAP, NO Ph chromosome (-), NORM plts, and anemia W/O nRBCs.
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| What are symptoms of CML? | -Blasts/promyelocytes in BM
-HIGH Eos and Basos
-Pseudo Pelger-Huet
-Low LAP
-ph chromosome
-INC plts
-Anemia with nRBCs
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| What are symptoms of Leukamoid reaction? | -Dohle bodies
-Toxic granulation
-Vacuoles present
-NORMAL Eos and Basos
-High LAP
-NO ph chromosome
-Normal plts
-Anemia w/o nRBCs
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| What is Polycythemia vera? | Disease where the BM developed in uncontrolled way = Panmyelosis, 100% cellularity. Inc RBCs, WBCs, PLTs. Splenomegaly is common. Stem cells are >> sensitive to erythropoetin. They are morphologically typical.
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| How would you diagnose Polycythemia vera? | RBC mass of 36mL/kgWomen and 32mL/kgMen. Normal O2 saturation(lower = make more RBCs to compensate for less conc.), Splenomegaly. IF 1/3 tests are missing,compens 2/3: Thrombocytosis >400, Leukocytosis(>12)w/oFever,Inc.LAP,B12,unbound B12 binding capacity
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| How would BM look like in Polycythemia? | HUGE megakaryocytes, and a bunch of different precursors like RBC, lymphocytes, etc. You see expansion of ALL the different populations not just RBCs. The cells look normal for the most part.
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| What is the prognosis of Polycythemic vera? | Stable phase long time(RBCs live long=accum in bld,high#s long time more mutations),Spent phase=stopRBC prdctn sake of cancer prvntn.OR prgress to AcuteLeukemia=splenomegaly/hypersplen&pancyto.Also see: BM fibrosis,splenomegaly,Anemia w/teardrop poikcytes
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| What is a treatment of Polycythemic vera? | Prophylactic phlebotomy
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| REVIEW: What is Pancytopenia? | Dec # of cells across the board
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| REVIEW: What is Splenomegaly? | Enlarged spleen
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| What is Essential Thrombocytopenia Disorder? | Appears after 60yrs, inc megakaryopoiesis. in BM: Megakaryocyte hypercellularity, INC in all precursors, Reticulin fibers maybe increased
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| How is Essential Thrombocythemia diagnosed? | -Thrombocytosis >600
-Hgb <13 (normal RBC mass)
-Sufficient Iron stores
-Ph chrom negative
-BM is not fibrotic
-No reason to suspect rctiv thrombocytosis (not recovering from cancer or other treatment)
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| What is found in peripheral blood of Essential Thrombcythemia? | >>plts
>>change in plt size/shape, granularity, shape
-Normal WBCs in morphology and #
-RBCs norm/norm unless there was bleeding (if there was, the RBCs will change to INC of MCV and polychromasia)
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| What is prognosis of Essential Thrombocytopenia? | Plts occlude small vessels=Erythromelalgia, Trnasient ischemic attacks, seizures, cerebral/myocardial infarction.
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| What is treatment of Essential Thromb.? | Suppression of Plt production through radiation or drugs (but inc risk of leukemic transformation)
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| What are Myelodysplastic Syndromes in general? | De novo or Treatment-related to Alkylating agents, Topoisomerase II inhibitors, Radiation.
As oppose to Myeloproliferative where cells are made over and over, Myelodysplastic is where the cell doesn't make cell cycle checkpoint. MUCH less mature nuclei.
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| If you see LOTS of plts, what disease is it? | Essential thrombocythemia
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| If you see LOTS of RBCs, what disease is it? | Polycthemia vera
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| If you see LOTS of Nuetrophils, what is it? | CML
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| What makes CML NOT acute but chronic? | <20% blasts
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| If there's LOT of cells, you either have...? | Acute leukemia or chronic leukemia, and look at what cell is most prevalent, so it's prob the one that causes the disease.
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| Myelodysplastic syndromes are experienced in? | Older adults
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| What is found in PB of Myelodysplastic Syndromes? | DYSERYTHROPOIESIS. *Oval macrocytes w/normal B12/folate* HypochromMicrocytes(w/normIronlevels), Dimorphic RBC, Poikylo, BasophilStippling, HowellJolly, Siderocytes, Plts w/Abnormal form
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| What are the 3 diff disorders where oval macrocytes? | B12, Folate defiiencies, and dysplastic disesae=myelodysplasia
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| What are BM changes in Myelodysplastic syndromes? | -RBC precursors w/mult.nuclei or abnormal shapes(lobes/buds)
-NuclearFragments
-Basophilic stippling
-Heterogenous staing of cytoplasm
-Ringed sideroblasts
-Megaloblastoid changes
-Persis.Basophilia in matureWBCs
-1ary gran large
-2ary gran absent
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| What are diseases of Myelodysplastic Disorders? | -Refractory anemia
-RA w/ ringed sideroblasts (RARS)
-RARS w/ unilineage dysplasia
-RARS w/multilineage "
-Refract.Cytopen w/multilinDysplasia
- " anemia w/ExcessBlasts
-5q-syndrome(del)
ALL LOW CELL COUNTS=anemias w/no resp to IRON TREAT.
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| What are two types of Refrac anem w/excess blasts? | Type 1 = 5-9% blasts
Type 2 = 10-19% blasts
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| How do you know it's dysplasia in the first place? | Defintion: Anemia due to decreased production and you can see Iron and B12 are normal.
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| Describe each. | LOOK at the ppt
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| What is the prognosis of Myelodysplastic Disorders? | De novo is 1-6yrs, Therapy related is 10months, and 50-80% progress to AML
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| What is treatment for Myelodysplastic Disorders? | Palliative: Transfusions (RBC or Plts), or Colony stimulating factor. Cure: BM transplant, Cord blood, Low intens transplant(graftvsleukemia)
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| What are complications of treatment? | Graft vs host disease, Rejection of graft
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| What is Acute Myeloid Leukemia? AML | Everything is all messed up and nothing is created the way it should.
>20% blasts in PB or BM
PPl are sicker than those w/ leukemias or chronic
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| What are the WHO classifications of AML? | -AML w/recurrent cytogenetic translocations
-AML w/multilineage dysplasia
-AML&myelodysplastic syndrome, therapy-related
-AML not otherwise catergorized
-Acute Biphenotypic Leukemia
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| What is AML w/recurrent cytogenetic translocations? | AML w/ t(8;21)(q22;a22), AML (M2)
Acute promyelocytic leukemia (M3) which is AML w/ t(15;17)(q22;q11-12) and variants
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| What is Acute promyelocytic leukemia (M3)and symptoms? | Females>males, young adults and elderly, sudden sever progression. Bleeding due to release of procoagulant/DIC, Heparin can be used to prevent this.
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| What is the morphology of Acute promyelocytic leukemia (M3)? | Promyelocytes predominate in peripheral blood, nucleus is delicate/folding, Auer rods, Two morpholic variants: Hypergranular and hypogranular
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| What are stains for Acute promyelocytic leukemia (M3)? | -Myeloperoxidase +
-Choloracetate +
-Nonspecifci esterase -
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| What is treatment for Acute promyelocytic leukemia (M3)? | High does Vit A for maturation. Cells apoptose... but there is Retinoic acid syndrome.
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| What is AML with MultiLineage Dysplasia? | With or W/out myelodysplastic syndrome. Includes: AML+Dysplasia (>50% cells of 2 or more myeloid lines in pre-treatment), may occure De novo or following MDS or MDS/MPD. Mainly in elderly and rare in kids
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| What can AML with mulitlineage be presented with? | -Sever pancytopenia
-Dysregranulopoeisis, dyerythropoesis, dysmegakaryopoieises
-Blasts + for CD34, and pan-myeloid markers (CD13 and CD33)
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| What is prognosis of AML with multilineage? | Multilin has adverse effect on remission
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| What is AML and Myelodysplastic syndrom that's Therapy related? | History shows CHEMOTHERAPY! (alkylating agent/radiation therapy)
-all myeloid cells lines affected
-Dyserythropoiesis
-Ringed sideroblasts in 60% of cases (1/3 in excess of 15% erythroids)
-Types: M2, M4, M5, M6, M7 (don't need to know this)
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| What is the prognosis of AML and Myelodysplastic syndrom that's Therapy related? | Poor response to therapy, poor survival
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| What is AML not otherwise categorized? | Don't need to know all the M's
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| What are two types of Lymphoproliferative Disorders and Lymphomas? | Disease of the cell making many copies of themselves BUT the blast number is <20%. (If MORE than 20, it's acute lymphocytic leukemia)(more than 20% myeloblasts=acute myeloblastic anemia)
-Hodgkin's Lymphoma
-Non-Hodgkin's Lymphoma
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| What is Hodgkin's Lymphoma? | Proliferation of Lymphocytes, Histiocytes, Plasma cells, eosinophils, Reed-sternberg cells.
-1/3 of all malignant lymphoproliferative disorders
-Common in children
-Usu. found in lymph node but also the lymph node chain
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| What is the prognosis of Hodgkin's lymphoma? | 80-90% remission, development of this disease maybe be linked to development of other cancers
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| What is Non-Hodgkin's lymphoma? CLL | B cell NEOPLASMS:
-Small cell lymphocytic Lymphoma (primarily involves nodes)
-Chronic lymphocytic leukemia (primarily involves peripheral blood or BM)
Essentially same as Hodgkins.
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| What are the B cell Neoplasms? | -Hairy Cell Leukemia
-Burkitt's lymphoma
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| What is Hairy cell leukemia? | -2% all leukemias,occurs middle age
-Insidious onset (weakness and lethargy bc they get anemic)
-Splenomegaly: Dry tap (reticuloedothelia infiltrates), Tartrate resist.acid phosphate(TRAP)+, Immunotyping:surface and cytoplasmic Ig +, PCA-1 +, PC-1 (-)
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| What is Burkitt's Lymphoma? | -High grade malignancy
-In children/adults/those with AIDs
-Large jaw mass
-Linked to EBV
-CD19,20,22 +
They get Mono
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| What is Acute Lymphocytic Leukemia? ALL | -Disease of Childhood (age 2-10, or elderly patients)
-Good prognosis in kids (90% remission rate, 60% cured) Adults (68-91% remission, cure 25-41%)
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| What is the presentation of ALL? | -50% patients have leukocytosis & lymphoblastsCSF
- (+/-) circulating lymphoblasts
-Typical findings:Neutropenia, Thrombocytopenia, Anemia
-Fatigue, Fever, Bleeding
-Enlarged lymph nodes, spleen or liver
-Bone pain with infiltration of leukemic cells
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| How else can you characterize ALL? | Immunotyping, 4 types
-Immature B cell (+ CALLa/CD10)
-Pre B-cell (- CALLa/CD10)
-T-cell
-B-cell
Further typing can be done by distinguishing between Bcell precursors and Tcell/matureBcell is clinically significant
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