Create
Learn
Share

IM - LEUKEMIAS & MYELOPROLIFERATIVE DISORDERS

rename
tonystep1's version from 2017-08-03 08:27

LEUKEMIA CLINICAL FEATURES

Question Answer
Acute lymphoblastic leukemia (ALL)Anemia and associated symptoms // Increased risk of bacterial infections (due to neutropenia) // Abnormal mucosal or cutaneous bleeding (due to thrombocytopenia // Splenomegaly, hepatomegaly, lymphadenopathy // Bone and joint pain (invasion of periosteum) // Testicular involvement // Anterior mediastinal mass
Acute myelogenous leukemia (AML) Anemia and associated symptoms // Increased risk of bacterial infections (due to neutropenia) // Abnormal mucosal or cutaneous bleeding (due to thrombocytopenia // Splenomegaly, hepatomegaly, lymphadenopathy // Bone and joint pain (invasion of periosteum) // Skin nodules
Chronic Lymphocytic Leukemia (CLL)Usually asymptomatic; CLL may be discovered on a routine CBC (lymphocytosis) // Generalized painless lymphadenopathy (lymph nodes are nontender) ,splenomegaly // Frequent respiratory or skin infections due to immune deficiency // In more advanced disease: fatigue, weight loss, pallor, skin rashes, easy bruising, bone tenderness, and/or abdominal pain
Chronic Myeloid Leukemia (CML)Usually asymptomatic at time of diagnosis-discovered on routine blood work // Constitutional symptoms-fevers, night sweats, anorexia, weight loss // Recurrent infections, easy bruising/bleeding, symptoms of anemia // Splenomegaly, hepatomegaly, lymphadenopathy
memorize

LEUKEMIA DIAGNOSIS

Question Answer
Acute myelogenous leukemia (AML)The WBC count is variable (from 1000/mm3 to l00,000/mm3) . // Significant numbers of blast cells (immature cells) in peripheral blood.// Bone marrow biopsy is required for diagnosis-replacement of marrow by blasts // Auer rods are seen inside the malignant cells
Acute lymphoblastic leukemia (ALL) The WBC count is variable (from 1000/mm3 to l00,000/mm3) . // Significant numbers of blast cells (immature cells) in peripheral blood.// Bone marrow biopsy is required for diagnosis-replacement of marrow by blasts //
Chronic Lymphocytic Leukemia (CLL)CBC-WBC: 50,000 to 200,000 // Absolute lymphocytosis-Almost all of the WBCs are mature, small lymphocytes. // Presence of smudge cells-leukemic cells that are "beaten up" in the blood // Bone marrow biopsy-presence of infiltrating leukemic cells in bone marrow
Chronic Myeloid Leukemia (CML)Marked leukocytosis-WBCs from 50,000 to 200,000 with a left shift toward granulocytes // Small numbers of blasts and promyelocytes // Eosinophilia // Peripheral smear-leukemic cells in the peripheral blood: myelocytes metamyelocytes, bands, and segmented forms // Decreased leukocyte alkaline phosphatase activity // Bone marrow biopsy: leukemic cells
memorize

TREATMENT OF LEUKEMIAS

Question Answer
Acute Leukemias Treatment of emergenciesBlood cultures, antibiotics for infections // Blood transfusion for anemia and platelet transfusion for bleeding, if necessary
Acute lymphoblastic leukemia (ALL)Aggressive, combination chemotherapy in high doses for several weeks // Once remission occurs, maintenance therapy is used for months or years // Bone marrow transplantation // More than 75% of children with ALL achieve complete remission Up to 50% of patients are cured.
Acute myelogenous leukemia (AML) Aggressive, combination chemotherapy in high doses for several weeks // Once remission occurs, maintenance therapy is used for months or years // This is more difficult to treat and does not respond as well to chemotherapy // Bone marrow transplantation gives the best chance of remission or cure
Chronic Lymphocytic Leukemia (CLL)Chemotherapy has little effect on overall survival, but is given for symptomatic relief and reduction of infection. Patients are often observed until symptoms develop // Fludarabine and chlorambucil have been shown to be of some benefit.
Chronic Myeloid Leukemia (CML) Chemotherapy may control symptoms before the acute phase develops // progressive disease with no cure // An alkylating (e.g. cyclophosphamide) agent or an antimetabolite (e.g. Methotrexate) is used to treat the chronic phase // Bone marrow or stem cell transplantation may be appropriate during the chronic phase because most cases eventually advance to an acute phase.
memorize

MYELOPROLIFERATIVE DISORDERS CLINICAL FEATURES

Question Answer
Polycythemia Vera1 . Symptoms due to hyperviscosity: headache, dizziness, weakness, pruritus, visual impairment, dyspnea 2. Thrombotic phenomena-DVT, CVA, myocardial infarction, portal vein thrombosis 3. Bleeding-GI or genitourinary bleeding, ecchymoses, epistaxis 4. Splenomegaly, hepatomegaly 5. HTN
Myelodysplastic Syndromesl . They are often asymptomatic in the early stages. Pancytopenia may be an incidental finding on a routine blood test. 2. They may present with manifestations of anemia, thrombocytopenia, or neutropenia.
Essential ThrombocytosisIt is primarily manifested by thrombosis (e.g. , CVA) , or paradoxically and less frequently, bleeding (due to defective platelet function). It is a disease with high morbidity but low mortality. // Other findings may include splenomegaly, pseudohyperkalemia, and elevated bleeding time. // Erythromelalgia is burning pain and erythema of the extremities due to microvascular occlusions.
Agnogenic Myeloid Metaplasia With MyelofibrosisThis condition refers to fibrosis of the bone marrow resulting in pancytopenia and extramedullary hematopoiesis. // massive splenomegaly is usually present. Other manifestations are secondary to pancytopenia-e.g. , fatigue, bleeding, infection.
memorize

MYELOPROLIFERATIVE DISORDERS DIAGNOSIS

Question Answer
Polycythemia VeraRule out causes of secondary polycythemia (e.g., hypoxemia, carbon monoxide exposure) // Elevated RBC count, hemoglobin, hematocrit (usually >50) // Thrombocytosis, leukocytosis may be present // Serum erythropoietin levels are reduced // Elevated vitamin B12 level // Hyperuricemia is common // Bone marrow biopsy confirms the diagnosis
Myelodysplastic SyndromesBone marrow biopsy typically shows dysplastic marrow cells with blasts or ringed sideroblasts. // CBC with peripheral smear -- Normal or mildly elevated MCV -- Low reticulocyte count -- Other abnormalities may include Howell-jolly bodies, basophilic stippling,nucleated RBCs, hypolobulated neutrophilic nuclei, and large, agranular platelets.// Cytogenic studies often reveal chromosomal abnormalities or mutated oncogenes.
Essential ThrombocytosisDefined as platelet count >600,000/mm3 // Peripheral smear shows hypogranular, abnormally-shaped platelets. // Bone marrow biopsy shows an increased number of megakaryocytes
Agnogenic Myeloid Metaplasia With MyelofibrosisTeardrop cells on peripheral smear are a hallmark feature. Large, abnormal platelets and immature myeloid cells are also present.//Bone marrow aspirate shows marrow fibrosis-if severe enough, it may be a "dry tap."
memorize

TREATMENT OF MYELOPROLIFERATIVE DISORDERS

Question Answer
Polycythemia VeraRepeated phlebotomy to lower hematocrit // Myelosuppression with hydroxyurea or recombinant interferon alfa (riFN-a)
Myelodysplastic SyndromesRBC and platelet transfusions are the mainstays of treatment // Erythropoietin may help to reduce the number of blood transfusions necessary // Granulocyte colony-stimulating factor can be an effective adjunctive treatment for neutropenic patients // Vitamin supplementation, particularly with vitamins B6, B1 2, and folate, is important given the large turnover of marrow cells // Pharmacologic therapies have variable results. // Bone marrow transplantation is the only potential cure.
Essential ThrombocytosisTreatment usually involves antiplatelet agents such as anagrelide and low-dose aspirin. // Hydroxyurea is sometimes used for severe thrombocytosis
Agnogenic Myeloid Metaplasia With MyelofibrosisTreatment is primarily supportive, involving multiple blood transfusions, erythropoietin, and sometimes splenectomy for palliative relief of painful splenomegaly.//Bone marrow transplantation is sometimes appropriate
memorize

Recent badges