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Pathoma
Blood neoplasm
| Question | Answer |
|---|---|
| Progenitor cell in BM that makes all blood cells is...? What is it's marker? | Hematopoietic CD34+ stem cell. Produces Myeloid stem cell (makes Erythroblast, Myeloblast, Monoblast, Megakaryoblast) or Lymphoid stem cell (makes B & T lymphoblasts) |
| What do the B lymphoblast and T lymphoblast make? | B lymphoblast makes a naive B cell-> makes plasma cells. T lymphoblast makes naive T cells-> CD8+ T cells, CD4+ T cells |
| What does myelolasts make? | Granulocytes (Neutrophils, Eosinophils & Basophils) |
| What kind of cells are released from BM into blood? | Mature cells are released from BM into blood |
| What is normal WBC count. What is low and high WBC count? | Normal WBC is 5000-10,000. Low WBC is called LEUKOPENIA. High WBC count is LEUKOCYTOSIS. Usually due to one type of WBC. |
| What is cause of NEUTROPENIA? | Drug toxicity (esp CHEMOTHERAPY affecting rapidly dividing cells in body including BM division) & Severe infection |
| How can such neutropenia be treated? | GM-CSF (granulocyte-monocyte colony stimulating factor) or GCSF (granulocyte-colony stimulating factor) are used to boost neutrophil count |
| What is cause of lymphopenia? | Immunodeficiency (DiGeorge syn; no 3rd/4th pharyngeal pouch). High cortisol state. Autoimmune destruction (SLE; mostly produce Ab against blood cells). Whole body radiation (most sensitive cells to radiation are LYMPHOCYTES, they die very quickly) |
| How does high cortisol state cause lymphopenia? | Cortisol or corticosteroids induce apoptosis in lymphocytes; eg cushing's |
| What are common causes of neutrophilic leukocytosis (left shift)? | Bacterial infection, Tissue necrosis, High cortisol state. During left shift, when immature precursor neutrophils are released into blood, they show decr Fc receptors (which help neutrop to recogn Ig for opsonin) |
| How does left shift affect opsonization? | Lots of immature neutrophils are made. They will have ineffect Fc receptors. Note that CD16 is marker for the decr Fc receptor. So both CD16 & Fc receptor will be decreased |
| How does high cortisol state cause neutrophilic leukocytosis? | Neutrophils which hang along blood vessels are released by cortisol into bloodstream; increasing neutrophil count |
| What are the important causes of monocytosis? | Chronic inflammatory states & Malignancy |
| What are the causes of eosinophilia? What mechanism mediates eosinophilia? | Allergic reactions, Parasitic infections, HODGKIN lymphoma. IL-5 causes eosinophilia to occur due to these 3 reasons |
| Basophilia is caused by...? | CML classically cause basophilia |
| Lymphocytic leukocytosis is mostly caused by...? | Viral infections & Bordetella pertussis (only bacteria that can cause lymphocyte incr), EBV infection |
| Why does Bordetella pertussis cause lymphocytic leukocytosis? | It produces Lymphocytosis promoting factor; blocks lymphocytes from getting into lymph nodes, so they stay in blood |
| What is cause and features of infectious mononucleosis? | Caused by EBV virus. Leads to LYMPHOCYTIC LEUKOCYTOSIS comprised of reactive CD8+ T cells. CMV is less common cause. Virus is transmitted by saliva (kissing disease in TEENAGERS) |
| Which tissues are primarily infected by EBV? | Oropharynx- causing sore throat. Liver- causing HEPATITIS. B cells also infected |
| What is immune response to EBV infection? | CD8+ T-cell response. Causes generalized LYMPHADENOPATHY (Cervical); will hyperplasia of PARACORTEX (where T-cells live). Splenomegaly (PERIARTERIAL LYMPHATIC SHEATH; PALS; T cell area). Incr white count w atypical lymphocytes |
| Why is IM called "monocytosis" | Bcos the appearance of the REACTIVE T cells look like monocytes (so a misnomer) |
| How is IM screened? | MONOSPOT TEST is used for screening. Detects IgM heterophile Ab. Turns +ve w/in 1wk after infection. -ve test suggests CMV as cause or too early testing (before <1wk) |
| How is definitive diagnosis of IM made? | Testing for EBV viral capsid antigen |
| What are heterophile antibodies? | These are Ab which have affinity for other RBCs of other animals. So IgM due to IM can bind sheep, horse etc RBCs. It's the IgM heterophile Ab that can be detected during monospot test |
| What are complication of IM? | Increased risk for splenic rupture due to splenomegaly (so patient is advised to avoid all contact sport for 1yr). Rash if exposed to penicillin. Dormancy of virus in B cells (so risk of recurrency and risk of lymphoma) |
| What is cause of Acute leukemia? | Disruption in the ability for the cell to mature; so Myeloblast or lymphoblast looses ability to become a more mature cell, so the myeloblast/lymphoblast piles up |
| What is definition of Acute leukemia | It's neoplastic proliferatn of blasts. Defined as accumulation of >20% blasts in bone marrow. Blast "crowd-out" normal hematopoiesis-- result in "acute" presentatn w anemia, thrombocytopenia & neutropenia |
| Describe the blasts. | Blasts usually enter blood, resulting in high WBC. Blasts are large, immature cells, often w punched out nucleoli on blood smear |
| How is blasts in blood be identified? | If there is accumul. of myeloblast, it's called AML. If there is accumul of lymphoblast, it's ALL. Lymphoblast is +ve ONLY for tDt (DNA polymerase) in the nucleus. Myeloblast will b +ve for MPO (myeloperoxidase) |
| How can myeloperoxidase be tested in myeloblast in blood | Detected in 2 ways: chemical study to detect presence. View MPO under microscope; bcos it can crystalize to form AUER RODS. So AUER RODS in microscope indicate AML |
| Describe ALL | Neoplastic accumul of lymphoblasts. +ve nuclear staining for TdT (DNA polymerase in nucleus). Can be T or B ALL based on surface markers. TdT is absent in myeloid blasts & mature lymphocytes. |
| What are features and association of ALL? | Most common in children. Assoc w Down syndrome (after age 5). Excellent response to CHEMO. But requires prophylactic treatment to SCROTUM (Testes-blood barrier blocks chemo) & CSF (BBB blocks chemo) |
| What is the most common ALL type. What is its markers? | B-ALL is most common. Lymphoblasts classically express CD10, CD19, CD20 |
| What is prognosis of ALL based on? | Prognosis based on cytogenetic abnorm. t(12:21) has a good prognosis- more commonly seen in kids. t(9:22) has a poor prognosis- more commonly seen in adults (Ph+ ALL). |
| t(9;22) is also seen in...? | CML |
| T-ALL is categorized by ... | Lymphoblasts that express markers ranging from CD2 up to CD8. Blasts do not express CD10 |
| Which physical feature does T-ALL present with? | Presents as a mediastinal (T..hymic) mass in a T..teenager. Because it's in lymphoid tissue, it called Acute lymphoblastic LYMPHOMA |
| Describe AML | AML is a neoplastic accumulation of myeloblasts. Stains +ve for MPO (Auer rods crystals). AML is seen in OLDER ADULTS 50-60yrs. Subclassified as Cytogenetic abnorm (predominant mechanism of classifying), lineage of myeloblast, surface markers |
| AML characterized by t(15;17) is called...? | Acute Promyelocytic Leukemia. t(15;17) causes Retinoic Acid Receptor disruption; promyelocytes accumulate. |
| What condition does Auer rods in promyelocytes cause? | Risk for DIC. Because auer rods can activate the coagulation cascade, so Acute Promyelocytic Leukemia is an EMERGENCY, due to risk of DIC |
| What causes APL blasts to mature? | ATRA (All Trans Retinoic Acid) causes blasts to mature. ATRA binds RAR receptor & cause blast to mature to neutrophils (eliminates the leukemic burden |
| What are the types of AML that can occur? | Erythroblastic AML, Monoblastic AML, Megakaryoblastic AML, Acute MONOCYTIC Leukemia |
| What is Acute monocytic leukemia? | Proliferation of monoblasts; lack MPO. Blasts classically infiltrate GUMS |
| What is Acute megakaryoblastic leukemia? | Proliferation of megakaryoblasts. Lack MPO. Associated w Down syndrome (before age 5) |
| Which cancer is Down syndrome patients associated with? | Acute Leukemia; esp Acute Megakaryoblastic Leukemia b4 age 5. OR ALL after age 5 |
| How can AML arise? | AML may arise from pre-existing dysplasia. Prior exposure to alkylating agents or radiotherapy |
| Which syndrome do AML patients get? | Myelodysplastic syndromes: cytopenia w hypercellular bone marrow (dont get out to blood). Abnormal maturatn w incr blasts (<20%). Most patients die from infection/bleeding. 20% progress to acute leukemia |
| What is chronic leukemia? | Neoplastic proliferation of MATURE lymphocytes; ie CD8+ & CD4+ T cells in the BLOOD. Will have high WBC count. Usually insidious (gradual) in onset & seen in older adults |
| Describe Chronic Lymphocytic Leukemia? | Neoplastic proliferatn of naive B-cells. Cells co-express CD5 & CD20. Incr lymphocytes & smudge cells seen on blood smear |
| How does CLL associate with lymph nodes? | CLL involves lymph nodes; (although it's a leukemia). Causes generalized lymphadenopathy. Now called SMALL LYMPHOCYTIC LYMNPHOMA, when lymph nodes are involved in CLL |
| What are complications of CLL? | Hypogammaglobulinemia (bcos CLL are B-cell which normally bcome plasma to secrete Ab). Autoimmune hemolytic anemia (the few Ab made targets RBCs). Transformation to diffuse large B-cell lymphoma |
| What is Hairy cell leukemia? | Chronic neoplastic proliferation of mature B cells. Characterized by hairy cytoplasmic processes. Cells are positive for TRAP (Titrate Resistant Acid Phosphatase) |
| What are clinical features of hairy cell leukemia? | Splenomegaly (red pulp; other chronic Leuk proliferate in white pulp). Dry tap (due to BM fibrosis) w BM aspiration. Lymphadenopathy (other chronics have enlarged lymph nodes) is usually absent |
| What is mnemonic for Hairy cell leukemia? | TRAP- TRAP rxn. Cells get TRAPed in RED PULP. Cells get TRAPed in BM fibrosis. TRAPped cells are NOT able to reach lymph nodes (so no lymphadenopathy) |
| What is treatment of hairy cell leukemia? | Excellent response to 2-CDA. It's an Adenosine deaminase inhibitor. Adenosine accumulates to toxic levels in neoplastic B cells. Note: Adenosine is part of purine degradatn pathway |
| What is Adult T-cell Leukemia Lymphoma (ATLL) | This is neoplastic proliferation of mature CD4+ T cells. Associated with HTLV-1 (Japan & Caribbean) |
| Clinical features of ATLL? | Rash, Generalized LAD with Hepatosplenomegaly (bcos mature lymphocytes go to lymph node & spleen). Lytic bone lesions w hypercalcemia. |
| Which 2 blood cancers cause lytic bone lesions? | Multiple Myeloma & ATLL. Cause punched out lesions. The RASH will indicate it's ATLL instead of multiple myeloma |
| What is mycosis fungoides? | Chronic leukemic process. Cause neoplastic proliferatn of mature CD4+ T cells. Aggregates of neoplastic T cells in epidermis are called Pautrier microabscesses |
| In mycosis fungoides, when cells spread to the blood, it's called? | Sezary syndrome. Characteristic lymphocytes w cerebriform (cell looks like brain with lobes) nuclei r seen in blood smear |
| Neoplastic accumulation of mature myeloid cell is called...? | MYELOPROLIFERATIVE DISORDER: If incr RBC- Polycythemia vera. Incr granulocyte- CML. Inr megakaryocytes- Essential thrombocytemia |
| Which cells are increased in myeloproliferative disorder | Essentially all mature myeloid cell types are increased. However, the disease is named based on the PREDOMINANT cell |
| What are features of neoplastic proliferation of mature cells of myeloid lineage? | Disease of late adulthood. Results in high WBC count with hypercellular BM |
| What are complication of myeloproliferative disorder? | Increased risk for hyperuricemia & gout (due accum of purine as nucleus are being removed to make mature RBC. Also due to incr death of granulocyte). Progression to marrow fibrosis. Transformation to acute leukemia |
| What are characteristics of CML? | Neoplastic proliferation of mature myeloid cells; esp granulocytes. BASOPHILS are characteristically increased. SPLENOMEGALY is common |
| What is cause of CML? | t(9;22). Causes BCR-ABL fusion w incr TYROSINE KINASE ACTIVITY (signal transducer (oncogene); so drives overproduction of the neoplastic cells). |
| What is treatment of CML? | First line of treatment= IMATINIB. Blocks tyrosine kinase activity |
| What are the 3 phases of CML? | Chronic phase, Accelerated phase, Transformation phase (where they devlop acute leukemia). ENLARGING spleen suggests accelerated phase of disease. ENLARGED spleen means chronic phase |
| Transformation of CML to acute leukemia is... | Worst complicatn of CML. Transformation can lead to AML (2/3) or ALL (1/3). Mutation is in a pluripotent stem cell |
| In CML, which stem cell has the mutation? What does this mean? | Hematopoietic stem cells. This means that although CML is a myelogenous leukemia, the mutated hematopoietic stem cell can cause TRANSFORMATION to either AML or ALL |
| How can CML be differentiated from a leukemoid reaction? | CML granulocytes r LAP (Leukocyte Alkaline Phosphatase) -ve. CML is assoc with increased BASOPHILS. CML granulocytes exhibit t(9;22). Leukemoid rxn is due to ACUTE INFECTION, causing overproduction of granulocytes; LAP will be +ve, bcos fights da infectn |
| What are the features of polycythemia vera? | Neoplastic proliferation of mature myeloid cells, esp RBCs. Granulocytes & platelets are also incr. Assoc w JAK2 KINASE MUTATION (**JAK & VERA**) |
| What are clinical features of polycythemia vera? | Blurry vision & headache. Incr risk of venous thrombosis (Budd-chiari syndrome of hepatic vein). Flushed face due to congestion. Itching after bathing (due to excess mast cell's histamine) |
| What is most common cause of Budd-Chiari syndrome? | Polycythemia vera. Affects HEPATIC VEIN; cause infarct of liver |
| What is treatment for polycythemia vera? | PHLEBOTOMY is DOC. 2nd line treatment is HYDROXYUREA. Without treatment, death occurs within 1yr |
| How can polycythemia vera be distinguished from reactive polycythemia? | PV- SaO2 is normal, EPO is decr. In RP due to lung disease, SaO2 is low, EPO is incr. In RP due to ectopic EPO production, EPO is high, SaO2 is normal (since there is no hypoxemia |
| What are features of Essential Thrombocythemia? | Neoplastic proliferation of mature myeloid cells, esp platelets. RBC & granulocytes are also involved. Assoc w JAK2 kinase mutation |
| Besides, essential thrombocythemia, what else is associated with lots of platelets? | Fe-deficiency anemia- also cause incr in platelets |
| What are the symptoms of essential thrombocythemia? | Incr risk of bleeding (underfnx( and/or thrombosis (overfnxing). Rarely progress to marrow fibrosis or acute leukemia. No significant risk for hyperuricemia or gout |
| What is myelofibrosis? | Neoplastic proliferation of mature myeloid cells, esp megakaryocytes. Associated with JAK2 kinase mutation. Megakaryocytes produce EXCESS PDGF. Results in marrow fibrosis |
| What are clinical features of myelofibrosis | SPLENOMEGALY due to EXTRAMEDULLARY HEMATOPOIESIS. Leukoerythroblastic smear. Incr risk of infection, thrombosis and bleeding; bcos BM is not able to properly make WBC, RBC, platelets |
| What is the leukoerythroblastic smear? | RBCs made in marrow, marrow have RETICULIN GATES. Prevent immature cells from exiting until they r small enough. This doesn't occur in extramedul hematopoiesis- so smear will be leukoerythroblastic (immature WBC & RBCs in smear) |
| What RBC shapes may form in myelofibrosis? | Tear-drop cells. Bcos the RBCs stretch as they try to squeeze out of myelofibrotic marrow |
Created by:
djkdarko