Term
| What is the MOST COMMON cause of agranulocytosis? |
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Definition
| Complete absence of neutrophils in the peripheral blood is almost always d/t drug rxn. ALL tx: adults: combo chemo including daunorubicin, vincristine, predinsone, and asparaginase. If pts have philadelphia chromosome, add imatinib. Then maybe chemo plus bone marrow transplant |
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Term
| What does the blood show w/agranulocytosis d/t drug rxn? |
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Definition
| In these cases, the bone marrow shows an almost complete absence of myeloid precursors, w/other cell lines undisturbed. |
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Term
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Definition
| This is immune neutropenia associated w/seropositive nodular RA and splenomegaly. |
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Term
| What are the 3 MOST COMMON types of infection w/neutropenia? |
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Definition
| The 3 most common are septicemia, cellulitis, and pneumonia. |
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Term
| How do you tx neutropenia? |
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Definition
| You tx by 1) removing causitive agent, and 2)broad spectrum abx. |
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Term
| What is the MOST COMMON genetic abnml w/polycythemia vera? This is also present in essential thrombocytosis and other myeloproliferative disorders. |
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Definition
| A mutation in JAK2 has been demonstrated in 95% of cases. |
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Term
| 50 yo m presents w/ha, dizziness, tinnitus, blurred vision, fatigue, generalized pruritis, and frequent epistaxis. hct is 56, hgb is 19, rbc is increased, wbc is increased, platelets are 900,000, mcv is nml mchc is nml, mch is nml, rdw is nml, retics are nml. Basophils and eosinophils are increased, B12 levels are increased and serum erythropoietin is low. You get to perform a dre, which shows engorged rectal vv. Spleen is palpable. How do you confirm your dx, what complications are you worried about and how do you tx? |
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Definition
| This clinical scenario suggests polycythemia vera. Confirm dx w/positive finding of JAK2 mutation. Tx of choice is phlebotomy. The major complication is thrombosis, and bleeding can also occur. |
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Term
| What is the major cause of morbidity and mortality w/polycythemia vera? |
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Definition
| The major cause of morbidity and mortality is arterial thrombosis. |
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Term
| What is the MOST COMMON clinical problem w/essential thrombocytosis? This is also the most severe complication of polycythemia vera. |
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Definition
| The MOST COMMON clinical problem is thrombosis. |
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Term
| 55 yo f pt presents w/erythromlelalgia. hct is 40, hgb is 13, rbc is nml, wbc is sl elevated, platelet ct is 2,000,000, mcv is nml, mchc is nml, mch is nml, rdw is nml, and reticulocyte ct is nml. Large platelets are found on peripheral blood smear. What other test might you run, what complication are you concerned about, and how do you tx? |
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Definition
| This clinical scenario describes essential thrombocytosis. JAK2 mutations are found in 50% of cases. Thrombosis is the main source of morbidity. Tx of choice is hydroxyurea and tx goal is to keep platelets below 500,000. |
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Term
| Teardrop poikilocytosis is indicative of what disorder? |
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Definition
| This is indicative of myelofibrosis. |
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Term
| 58 yo m pt c/o fatigue and abdominal fullness. PE reveals hepatomegaly remarkable splenomegaly. hct is 30 and hgb is 10. rbc are decreased, wbc is elevated, platelets are 300,000, mch is nml, mchc is nml, mcv is nml, rdw is increased, reticulocytes are nml. Smear shows poikilocytosis and numerous tear-drop forms in red cell line. Nucleated rbc are present and giant degranulated platelet forms are seen. Bone marrow aspiration results in a dry tap. What do you dx and how do you tx? |
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Definition
| This clinical scenario suggests myelofibrosis. Pts w/mild dz may require no tx or occaisonal transfusion support. Lenalidomide can be used. |
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Term
| What triad indicates myelofibrosis? |
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Definition
| The triad consists of 1)teardrop poikilocytosis, 2) leukoerythroblastic blood, and 3) giant abnml platelets. |
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Term
| What is indicated by presence of a philadelphia chromosome? |
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Definition
| This 9q-22q translocation indicates cml. |
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Term
| What is indicated by bcr/abl gene? What test reveals this gene? |
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Definition
| This gene, found in pcr, indicates cml. |
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Term
| What is the median age of cml? |
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Definition
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Term
| 55 yo f presents w/fatigue, night sweats, low-grade fever, abdominal fullness. Pe reveals enlarged spleen and sternal tenderness. hct is 38, hgb is 13, rbc is nml, wbc is elevated to 150,000, platelets are 450,000, mcv nml, mch nml, mchc nml, rdw is nml, basophilia and eosinophilia are present. No nucleated rbcs are present. Bone marrow aspiration reveals hypercellular left-shifted myelopoiesis, with myeloblasts composing 3% of marrow cells. 3% blasts are present in blood. What do you suspect and how do you tx? |
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Definition
| This clinical scenario indicates cml. Tx is imatinib mesylate, 400 mg po qd. The only proven curative tx is allogenic bone marrow transplantation, but this is performed in pts under 40 yoa, in whom dz is not well controlled or who have entered accelerated dz phase. |
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Term
| Nausea, periorbital swelling, edema, rash, and myalgia are the most common complications of what med? |
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Definition
| These are the most common complications of imatinib mesylate, tx for cml. |
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Term
| What is the usual cause of myelodysplastic syndromes? |
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Definition
| These are usually idiopathic. |
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Term
| Abnml in chromosomes 5 and 7 are indicative of what disorder? |
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Definition
| These chromosomal abnml indicate myeloblastic syndromes. |
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Term
| 60 yo m pt presents w/fatigue, fever, weight loss, pallor, and bleeding. hct is 30, hgb is 10, rbc is decreased, wbc is sl reduced, platelets are 150,000, mcv is increased, mch is nml, mchc is nml, rdw is increased, retics are nml. Neutropenia is present and bilobed nucleus is present in neutrophils. Hypogranular platelets are present. Macro-ovalocytes are seen in peripheral blood smear. Bone marrow is hypercellular, and erythroid hyperplasia is present. Prussian blue stain reveals ringed sideroblasts. Dwarfed megakaryocytes w/unilobed nuclei are present. What do you suspect, what genetic abnml do you look for, and how do you tx? |
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Definition
| This clinical scenario is indicative of myelodysplastic syndrome. If 5q-cytogenetic abnml is present, tx of choice is lenalidomide. Otherwise, tx w/rbc transfusions. Azacitidine is tx of choice if pt has increased blsts in bone marrow. epo can be tried. Allogenic stem cell transplantation is the only curative tx, but role is lmt by advanced age of most pts. |
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Term
| What is the cause of most acute leukemias? |
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Definition
| Most arise from no clear cause. |
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Term
| What type of leukemia involves a chromosomal translocation, which creates a fusion gene (pml-rar(alpha)), which blocks cell differentiation and maturation? |
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Definition
| This typifies acute promyelocytic leukemia. |
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Term
| What comprises 80% of the acute leukemias of childhood, with peak incidence between ages 3 and 7? |
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Definition
| This age group typically presents w/ALL. |
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Term
| What leukemia has a mean presentation of age 60 yrs with increasing incidence with advancing age? |
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Definition
| This age is typical of AML. |
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Term
| F pt has 2 day hx of gingival bleeding, epistaxis, cellulitis, and pain in R tibia. Pt is pale, has purpura and petechiae, hepatomegaly, spenomegaly, and lymphadenopathy. CBC shows hct 20, hgb 6, decreased rbc, decreased wbc, platelets of 50,000, nml mcv, nml mchc, nml mch, increased rdw, nml reticulocytes. Peripheral smear shows 25% blasts. What further tests do you want to run, what do you suspect, and how do you tx? |
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Definition
| This clinical scenario describes Acute leukemia. The main way to differentiate between AML and ALL is age of the pt. You should run genetic testing. AML tx: anthracycline plus cytarabine followed by chemo and autologous/allogenic transplantation. APL tx: anthracycline plus all-trans-retinoic acid followed by chemo and autogolous/allogenic transplantation. ALL tx: combo chemo, followed by bone marrow transplant and cns prophylaxis to prevent meningeal sequestration of leukemic cells. |
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Term
| ALL is a malignancy of which cell line? |
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Definition
| This is a malignancy of stem cells |
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Term
| The hallmark of which leukemia is pancytopenia? |
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Definition
| This is the hallmark of acute leukemia. |
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Term
| Auer rods are found in what type of leukemia? |
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Definition
| These fused lysosoal granules are seen in wbc cytoplasm only in myeloid leukemia. |
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Term
| Differentiate between autologous and allogenic bone marrow transplants. |
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Definition
| The first is a transplant of the pt's own marrow to themselves; the second is a transplant from a donor with a similar bone marrow type. |
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Term
| What are the 3 requirements for remission? |
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Definition
| The 3 requirements are: 1) return of blood smears to nml smear. 2) nml bone marrw w/o blasts (nml 10-20%). 3)nml clinical status (any abnml not d/t ilness). |
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Term
| What is the goal of tx in leukemia? |
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Definition
| The goal of tx is remission. |
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Term
| What type of cells are involved w/cll? |
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Definition
| This is a malignancy of b-lymphocytes, in which injury to the dna results in uncontrolled growth, resulting in immunoincompetent cells. |
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Term
| Which malignancy is not associated w/xrt/chemicals? |
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Definition
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Term
| Which is more aggressive: cll or prolymphcytic leukemia? |
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Definition
| Prolympocytic is more aggressive. |
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Term
| How is cll most commonly discovered? |
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Definition
| This is typically an incidental discovery- lymphocytosis. |
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Term
| The Rai system is used for what malignancy? |
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Definition
| This determines tx for cll. |
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Term
| What is the hallmark of cll? |
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Definition
| The hallmark is isolated lymphocytosis. |
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Term
| Hypogammaglobinemia is associated w/which malignancy? |
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Definition
| This is associated w/cll. |
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Term
| A 65 yo f w/UTI has the following CBC results: hct 40, hgb 13, rbc nml, wbc 20,000 (elevated), platelets 300,000, nml mcv, nml mch, nml mchc, no reticulocytosis, nml rdw. wbc dif shows lymphocytosis. Hypogammaglobulinemia is present. Bone marrow aspiration reveals infiltration w/sm lymphocytes. What do you suspect and how do you tx? |
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Definition
| This clinical scenario indicates cll. Early dz requires no tx; progressive tx requires *combination regimen* w/ xrt/chemo, monoclonal abs, and nucleoside analogs. For refractory cases, allogenic transplant could be curative. |
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Term
| What leukemia is a malignancy of the bone marrow? |
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Definition
| This occurs w/cml, leading to overproduction of myeloid cells, esp abnml granulocytes. |
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Term
| Describe how myeloid cells change as cml progresses. |
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Definition
| Myeloid cells initially able to differentiate and maintain bone marrow function, eventualy transforming to more voertly malignant dz including blast crisis. |
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Term
| Which leukemia has a known association w/benzene? |
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Definition
| This association is present w/cml. |
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Term
| What is the most common physical finding w/cml? |
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Definition
| Splenomegally most common finding. |
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Term
| What leukemia involves blast crises? |
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Definition
| This occurs w/cml, w/increased bleeding and infection d/t bone marrow failure, anemia, etc. |
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Term
| What is the hallmark of cml? |
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Definition
| The hallmark is elevated wbc ct. |
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Term
| What leukemia involves bands? |
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Definition
| These immature neutrophils are found in CML. |
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Term
| When would you perform emergent leukapheresis w/cml? |
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Definition
| Perform this if symptoms of hyperleukocytosis appear. |
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Term
| What malignancy typifies hairy cell leukemia? |
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Definition
| This is typified by malignancy of b-lymphocytes, with infiltrates that interferes w/bone marrow function leading to faiilure. |
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Term
| What is the most common sign of hairy cell leukemia? |
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Definition
| The most common sign is weakness and fatigue from universal anemia. |
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Term
| 55 yo w m presents w/ progressive weakness and fatigue over the past 2 mos. PE reveals splenomegaly and hepatomegaly. CBC reveals hct 30, hgb 10, decreased rbc, decreased wbc, platelets 100,000, nml mcv, nml mchc, nml mch, nml reticulocytes. Pt has neutropenia and monocytopenia, and bone marrow tap is dry. What do you expect to see on blood smear and how do you tx? |
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Definition
| This clinical scenario typifies hairy cell leukemia, so you would expect to see hairy cells on peripheral smear. The mainstay of tx is cladribine (leustatin), given as continuous iv for 7 days. |
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Term
| What is the hallmark of non-hodgkin's lymphoma? |
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Definition
| The hallmark is painless lymphadenopathy. |
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Term
| What are the most common lad sites in non-hodgkin's lymphoma? |
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Definition
| Most common sites are neck, collarbone, axillae, and groin. |
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Term
| What translocation indicates burkett's lymphoma? |
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Definition
| This is indicated by t(8;14) |
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Term
| Which is more common: hodgkins or non-hodgkins lymphoma? |
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Definition
| Nonhodgkins is more common. |
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Term
| What malignant growth typifies non-hodgkin's lymphoma? |
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Definition
| This is typified by malignant growth of b or t lymphocytes w/in the lymph system. |
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Term
| 30 yo f c/o "bumps in her armpit." They do not hurt, and here cbc is nml. What do you suspect, how do you dx, and how do you tx? |
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Definition
| Painless lymphadenopathy is the hallmark finding of non-hodgkin's lymphoma. Bx the lymph nodes. CAT, MRI, PET, or Gallium could also be ordered to find how advanced the lymphoma is. Tx w/xrt/chemo(como, high dose), and bone marrow/stem cell transplant (don't wait for remission). |
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Term
| What is the intent of tx w/non-hodgkin's lymphoma? |
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Definition
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Term
| Reed-sternberg cells characterize what type of malignancy? |
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Definition
| These characterize hodgkin's lymphoma. |
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Term
| What malignancy is involved w/hodgkin's lymphoma? |
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Definition
| This involves malignant growth of cells, b-cell lymphocyte origin, in the lymph system. |
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Term
| In what malignancy is ebv a factor? |
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Definition
| This virus is a factor in hodgkin's lymphoma. |
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Term
| Which stage involves a single lymph node region OR one organ area outside the lymph node? |
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Definition
| This is Ann Arbor stage 1; early dz. |
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Term
| Which stage involves 2 or more lymph node regions on one side of the diaphragm? |
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Definition
| This is Ann Arbor stage 2; locally advanced dz. |
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Term
| Which stage involves dz in lymph nodes bo above and below the diaphragm? |
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Definition
| This is Ann Arbor stage III; advanced dz. |
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Term
| Which stage involves advanceent beyond lymph nodes and spleen AND spread to one or organs sucha s bone, marrow, skin, or liver? |
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Definition
| This is Ann Arbor stage IV; widespread dz. |
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Term
| What is the age distribution of hodgkin's lymphoma? |
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Definition
| This has a bimodal distribution: 25-30 and >55 yo. |
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Term
| How does hodgkin's lymphoma spread? |
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Definition
| This spreads along contiguous nodes. |
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Term
| 25 yo f presesnts w/ mass in neck that only hurts after drinking etoh-, weight loss, fatigue, night sweats, and generalized pruritis. CBC is nml, except for peripheral smear, which shows reid-sternburg cells. How do you dx, and how do you tx? |
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Definition
| This clinical scenario is indicative of hodgkin's lymphoma. Bx lymph node to dx. CT, MRI, PET, Gallium can be done to stage. Tx w/curative intent based on stage: combo chemo, marrow transplant, monoclonal abx, vaccine tx. |
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Term
| What malignancy characterizes multiple myeloma? |
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Definition
| This is characterized by malignancy of plasma cells, the part of the immune system producing abs. |
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Term
| What dz involves replacement of bone marrow, increased bone destruction, and paraprotein formation? |
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Definition
| These are 3 characteristics of multiple myeloma. |
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Term
| Bence jones protein is found in what malignancy? |
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Definition
| This protein is found in multiple myeloma. |
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Term
| Growth factors for angiogenesis and plasmacytomas are found in what malignancy? |
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Definition
| These growth factors are found in multiple myeloma. |
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Term
| 65 yo m c/o bone pain in back ribs, fatigue, weakness, cachexia, tachycardia, doe, and a recurrent head colds for the past 2 mos. CBC shows hct 30, hgb 10, low rbc, nml wbc, platelet ct 200,000, nml mcv, nml mchc, nml mch, nml retics, and presence of rouleaux. Neutrophils are nml. electrophoresis reveals serum paraprotein. Bone marrow shows infiltration by plasma cells. What further tests do you order and how do you tx? |
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Definition
| Paraprotein on serum protein electrophoresis indicates mm. Order urinalysis to see bence-jones protein, and mri/pet to stage. Tx w/high-dose xrt/chemo, monclonal ab tx, lenalidomide, plasmaphoresis, and tx hypercalcemia, then autologous stem cell transplantation. |
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Term
| What disorder is d/t clonal abnml of hemopoietic stem cell? |
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Definition
| This is the cause of myeloproliferative disorders. |
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Term
| What may be included in overproduction of bone marrow in myeloproliferative disorders? |
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Definition
| Overproduction may inlude wbcs, rbcs, and platelets. |
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Term
| What causes polycythemia vera? |
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Definition
| This is caused by overproduction of mature rbcs, which leads to increased viscosity adn increased hct. |
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Term
| Itching is a sign of what 2 hematopoeitic disorders? |
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Definition
| Hodgkins and polycythemia vera both have this symptom. |
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Term
| What is the cause of spurious polycythemia? |
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Definition
| This increase in rbcs is d/t dehydration |
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Term
| What is the hallmark of polycythemia vera? |
|
Definition
| The hallmark is an elevated hct. |
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Term
|
Definition
| This painful burning of the hands w/erythema is typical of essential thrombocytosis. |
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Term
| What causes myelofibrosis? |
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Definition
| This is caused by overprouction of collagen or fibrotic tissue in bone marrow, impairing bone marrow's ability to produce blood cells. |
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Term
| Which of malignancy/myeloproliferative dz have a prognosis of less than 5 yrs? |
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Definition
| Myelofibrosis: ~5 yrs. Myelodysplasia: ultimarely fatal. |
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Term
| Which malignancy and other blood disorders have a prognosis of 5-10 yrs? |
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Definition
| CML: 5 yrs CLL: over 6 yrs. NHL: 6-8 yrs. Hodgkins: <5-10, depending on stage. Multiple myeloma: 4-6 yrs. |
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Term
| Which malignancy and other blood disorders have a prognosis of over 10 yrs? |
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Definition
| Polycythemia: 11-15 yrs. Essential thrombocytosis: >15 yrs. AML: 70-80% complete remission. ALL: 90% complete remission. Hairy cell: >10 yrs. |
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