This action might not be possible to undo. Are you sure you want to continue?
Gangguan myeloproliferatif kronik :
gangguan akibat abnormalitas clonal hematopoetic stem cell yg didapat (akuisita), mengakibatkan peningkatan selularitas sum2 tulang yg diikuti peningkatan jumlah sel darah perifer termasuk trombositosis
_______________________________________ Hematopoetic stem cell : sel primitif sum2 tulang, asal dari seluruh jenis sel darah Clonal/Monoclonal : propagasi sel yang berasal dari sel progenitor tunggal
Classification of myeloproliferative disorders.
Myeloproliferative syndromes Polycythemia vera Myelofibrosis Essential thrombocytosis Chronic myeloid leukemia Myelodysplastic syndromes Acute myeloid leukemia
Polisitemia Vera Essentials of Diagnosis Increased red blood cell mass. Usually elevated white blood count and platelet count. Normal arterial oxygen saturation. Splenomegaly. .
Carboxyhemoglobin: smoking 3. Spurious polycythemia Primary polycythemia : PV Secondary polycythemia 1.Causes of polycythemia. Hypoxia: cardiac disease. Erythropoietin-secreting tumors (rare) . Renal lesions 4. high altitude 2. pulmonary disease.
In vitro. a finding not seen in normal individuals. the red blood cell mass should be measured to determine whether true polycythemia or relative (Spurius) polycythemia exists. True polisitemia ( elevated Red Cell Mass): primary or secondary. The hematocrit > 54% (M) or 51% in (F) When the hematocrit is elevated. The serum erythropoietin level is low. . Primary polycythemia (polycythemia vera) is a bone marrow disorder characterized by autonomous overproduction of erythroid cells. erythroid progenitor cells grow without added erythropoietin.
. normal red cell mass and low-plasma volume. Carboxyhemoglobin: smoking 3.Erythropoietin-secreting tumors (rare) Relative ("spurious") polycythemia presents in overweight and hypertensive (often on diuretic therapy). pulmonary disease.Secondary polycythemia 1. Renal lesions 4. Hmt <60%. high altitude 2. Hypoxia: cardiac disease.
Common complaints: headache. There is a high incidence of peptic ulcer disease.Sign and simptom Symptoms related to expanded blood volume and increased blood viscosity. 60% are men. dizziness. tinnitus. and fatigue. Spleenomegaly in 75% of cases but is nearly always enlarged when imaged Thrombosis is the most common complication of polycythemia vera and the major cause of morbidity and death. and the median age at presentation is 60 years. Polycythemia rarely occurs in persons under age 40 years. Physical examination: reveals plethora and engorged retinal veins. blurred vision.and epistaxis. Generalized pruritus. .
000. sometimes to counts exceeding 1. hypochromia. with panhyperplasia Iron stores are usually absent from the bone marrow Overproduction of uric acid may lead to hyperuricemia.000/uL. The white blood count is elevated to 10. The red blood cell mass is elevated. and poikilocytosis may result from iron deficiency Progressive hypersplenism may also lead to elliptocytosis.Laboratory Finding Hematocrit above normal. The bone marrow is hypercellular. at times greater than 60%.000-20. Microcytosis.000/uL The platelet count is variably increased. Platelet morphology is usually normal. Red blood cell morphology is normal. .
WhiteCount Hematocrit Platelet Count Red cellMorphology Chronic myeloid leukemia I N N or I N Myelofibrosis N or D or I N or I D or N or I Abn Polycythemia vera N or I I N or I N Essential thrombocytosis N or I N I N .Differensial Diagnosis Laboratory features of myeloproliferative disorders.
Anagrelide is a new drug for trombositosis Low-dose aspirin (81-325 mg daily) has been shown to reduce the risk of thrombosis. Myelosuppressive therapy : a high phlebotomy requirement.000/uL and neutrophil count < 2000/uL. One unit of blood (approximately 500 mL) weekly target: less than 45%. thrombocytosis. Hydroxyurea >> Alkylating because of less leukemogenic potential. .Treatment Phlebotomy. The usual dose is 500-1500 mg/d orally Target: platelets < 500. and intractable pruritus.
Polycythemia vera may convert to myelofibrosis or to chronic myelogenous leukemia.Prognosis Polycythemia is an indolent disease with median survival of 11-15 years. In approximately 5% of cases. the disorder progresses to acute myelogenous leukemia. which is usually refractory to therapy. . The major cause of morbidity and mortality is arterial thrombosis.
• Normal red blood cell mass.Essential Trombositosis Essentials of Diagnosis • Elevated platelet count in absence of other causes. . • Absence of Philadelphia chromosome.
hepatic. Venous thromboses may occur in unusual sites such as the mesenteric. slightly increased incidence in women. First sign is thrombosis.Symptoms and Signs Median age: 50-60 years. Some patients experience erythromelalgia(painful burning and erythema) Bleeding Splenomegaly is present in at least 25% of patients. Finding of an elevated platelet count. . or portal vein.
but giant degranulated forms seen in myelofibrosis are not observed. The bleeding time is prolonged in 20% of patients. The Philadelphia chromosome is absent to differentiate from chronic myeloid leukemia.000/uL). The bone marrow : increased megakaryocytes but no other morphologic abnormalities. The peripheral blood smear reveals large platelets. Red blood cell morphology is normal. The hematocrit is normal.000.000/uL. .Laboratory Findings Elevated platelet count is the hallmark of this disorder. WBC mildly elevated (not above 30. but with some immature myeloid forms. and may be over 2.
Differensial Diagnosis Laboratory features of myeloproliferative disorders. WhiteCount Hematocrit Platelet Count Red cellMorphology Chronic myeloid leukemia I N N or I N Myelofibrosis N or D or I N or I D or N or I Abn Polycythemia vera N or I I N or I N Essential thrombocytosis N or I N I N .
5-2 g/d. and in high doses congestive heart failure. mild anemia. and peripheral edema. Plateletpheresis. Vasomotor symptoms such as erythromelalgia and paresthesias respond rapidly to aspirin and eventually to control of the platelet count. . Anagrelide is highly effective in a dose of 2-4 mg/d but may cause headache.Treatment Standard therapy has consisted of hydroxyurea in a dose of 0.
The bone marrow may become fibrotic.Prognosis Essential thrombocytosis is an indolent disorder Average survival is longer than 15 years from diagnosis The major source of morbidity — thrombosis — can be reduced by appropriate platelet control. and a 1-5% risk of transformation to acute leukemia over 20 year . sometimes with splenic infarction. There is a 10-15% risk of progression to myelofibrosis after 15 years. and massive splenomegaly may occur.
• Other hematopoietic cell lines normal.IDIOPATHIC (AUTOIMMUNE) THROMBOCYTOPENIC PURPURA Essentials of Diagnosis • Isolated thrombocytopenia. • Normal bone marrow with normal or increased megakaryocytes. . • No systemic illness. • Spleen not palpable.
Destruction takes place in the spleen. .Patophysiology ITP: autoimmune disorder in which an IgG autoantibody is formed that binds to platelets. where splenic macrophages with Fc receptors bind to antibody-coated platelets. Splenectomy is highly effective therapy. Platelets are not destroyed by direct lysis.
oral bleeding. . An enlarged spleen should lead one to doubt the diagnosis. Adult form is usually a chronic disease and only infrequently follows a viral infection. and there is a 2:1 female predominance. Presenting complaint is mucosal or skin bleeding (epistaxis. Incidence between ages 20 and 50 years. purpura. menorrhagia.Symptoms and Signs ITP commonly in childhood Precipitated by viral infection and usually self-limited. and petechiae).
000/uL. . with a normal or increased number of megakaryocytes. which can be explained by bleeding or associated hemolysis (Evans's syndrome). with platelet counts that may be less than 10. Coagulation studies will be entirely normal.Laboratory Findings The hallmark of the disease is thrombocytopenia. Peripheral blood cell morphology is normal except that platelets are slightly enlarged (megathrombocytes). Other counts are usually normal except for occasional mild anemia. The bone marrow will appear normal.
Posttransfusion purpura 6. . Hematologic malignancies 3. SLE = systemic lupus erythematosus. Viral infections.Differensial Diagnosis Causes of thrombocytopenia. Drug-induced 4. Aplastic anemia 2. Hypersplenism 7. Idiopathic thrombocytopenic purpura 3. Secondary (CLL. AIDS 13. Disseminated intravascular coagulation 8. Hemangiomas 12. Hemolytic-uremic syndrome 10. Chronic alcoholism Nonmarrow disorders 1. SLE)1 5. Sepsis 11. Myelodysplasia 4. Bone marrow disorders 1. Thrombotic thrombocytopenic purpura 9. 1CLL = chronic lymphocytic leukemia. Megaloblastic anemia 5. Immune disorders 2. Liver failure 14.
000/uL. Prednisone works primarily by decreasing the affinity of splenic macrophages.000/uL. 1-2 mg/kg/d. An alternative steroid regimen is the use of high-dose dexamethasone.Treatment Initial treatment is with prednisone. CR 80% . Splenectomy is indicated if patients do not respond to prednisone. 40 mg/d for 4 days. the risk of bleeding is small with platelet counts above 50. Splenectomy is the most definitive treatment for idiopathic thrombocytopenic purpura. Splenectomy can be performed safely even with platelet counts less than 10. enhanced vascular stability. decrease antibody production. reduces the binding of antibody to the platelet surface.
Rituximab can produce good responses in some patients with refractory disease.Treatment High-dose intravenous immunoglobulin. azathioprine. is highly effective in rapidly raising the platelet count. Use for bleeding emergencies or situations. Danazol. 1 g/kg for 1 or 2 days. vincristine. and cyclophosphamide. . cyclosporine. Platelet transfusions are rarely used in the treatment of idiopathic thrombocytopenic purpura.
The major concern during the initial phases is cerebral hemorrhage. which becomes a risk when the platelet count is less than 5000/uL.Prognosis The prognosis for remission is good. Very low platelet counts caused fatal bleeding is rare. .
• Normal coagulation tests. • Elevated serum LDH. fever. • Microangiopathic hemolytic anemia. • Neurologic and renal abnormalities. .THROMBOTIC THROMBOCYTOPENIC PURPURA Essentials of Diagnosis • Thrombocytopenia. • Reduced level of ADAMTS13.
The most common drugs implicated are quinine and ticlopidine. to platelet agglutination and adhesion to endothelium.Introducing TTP is an uncommon syndrome with microangiopathic hemolytic anemia. drugs. Familial cases occur. or infections. but are rare. pregnancy. ADAMTS13. Deficiency of a von Willebrand factor-cleaving protease. The syndrome may also occur as a complication of bone marrow transplantation or the use of cyclosporine or tacrolimus. . thrombocytopenia. The syndrome is occasionally precipitated by estrogen use. TTP is seen primarily in young adults between ages 20 and 50 years. and a markedly elevated serum LDH. female predominance.
Symptoms and Signs Anemia. the patient appears acutely ill and is usually febrile. Pallor. Evans's syndrome is the combination of autoimmune thrombocytopenia and autoimmune hemol . bleeding. Patients may have abdominal pain and tenderness due to pancreatitis. Differential Diagnosis The normal values of coagulation tests differentiate TTP from DIC. confusion. purpura. Other conditions causing microangiopathic hemolysis (Table 13-18) should be excluded. On examination. and signs of neurologic dysfunction may be detected. petechiae. or neurologic abnormalities. Neurologic symptoms include headache. and alterations in consciousness from lethargy to coma. hemiparesis and seizures may occur. With more advanced disease. aphasia.
fibrinogen) are normal unless ischemic tissue damage causes secondary disseminated intravascular coagulation (DIC) present elevated fibrin degradation products may be seen. ADAMTS13 is usually absent during active disease. Coagulation tests (prothrombin time.Laboratory Findings Anemia Reticulocytosis and circulating nucleated red blood cells. Coombs test is negative. there may be thrombi in capillaries and small arteries. Pathologically. Increasing indirect bilirubin LDH is markedly elevated in proportion to the severity of hemolysis. . partial thromboplastin time. The hallmark is a microangiopathic blood picture with fragmented red blood cells Thrombocytopenia is invariably present and may be severe. with no evidence of inflammation.
TTP and hemolytic-uremic syndrome are not distinct disease entities. TTP characterized by more neurologic and severe thrombocytopenia and hemolytic-uremic syndrome with more renal failure.Differensial Diagnosis Evans's syndrome is the combination of autoimmune thrombocytopenia and autoimmune hemolytic anemia. . but the peripheral smear will show spherocytes and not red blood cell fragments.
Splenectomy performed in remission may prevent subsequent relapses. corticosteroids. and dextran has been used with success. Immunosuppression with drugs such as cyclophosphamide has also been effective. . 60 to 80 mL/kg of plasma should be removed and replaced with fresh-frozen plasma. Prednisone and antiplatelet agents (aspirin [325 mg three times daily] and dipyridamole [75 mg three times daily]) The combination of splenectomy.Treatment Plasmapheresis. Treatment should be continued daily until the patient is in complete remission.
but in 20% of cases the disease will be chronic and relapsing. Neurologic abnormalities are almost always completely reversed. 80 to 90 percent of patients now recover completely.Prognosis With plasmapheresis. . Most complete responses are durable.
This action might not be possible to undo. Are you sure you want to continue?
We've moved you to where you read on your other device.
Get the full title to continue listening from where you left off, or restart the preview.