Presentation is loading. Please wait.

Presentation is loading. Please wait.

NEOPLASTIC DISORDERS OF THE BONE MARROW Those that are not leukemias.

Similar presentations


Presentation on theme: "NEOPLASTIC DISORDERS OF THE BONE MARROW Those that are not leukemias."— Presentation transcript:

1 NEOPLASTIC DISORDERS OF THE BONE MARROW Those that are not leukemias

2 NEOPLASTIC BONE MARROW DISORDERS Neoplastic disorders of the bone marrow are grouped into 3 categories: Acute leukemias (to be discussed later) which represent clearly malignant neoplasms with a proliferation of blasts Myelodysplastic disorders which are characterized by peripheral pancytopenia Myeloproliferative disorders which are characterized by an increase in WBCs, and/or RBCs, and/or platlets Myelodysplastic and myeloproliferative disorders may be acute or chronic and may terminate in acute leukemias

3 MUTATIONS RESULTING IN NEOPLASTIC DISORDERS OF THE BONE MARROW

4 NEOPLASTIC BONE MARROW DISORDERS Myeloproliferative disorders are characterized by Panhypercellularity of the bone marrow Erythrocytosis, granulocytosis, and thrombocytosis in the peripheral blood One cell line is usually more prominent than the others. Hematologic classification is based on the most prominent cell line:

5 NEOPLASTIC BONE MARROW DISORDERS Prominent cell Disorder Myeloid series Chronic myelocytic leukemia (will be discussed later) Erythroid Polycythemia vera Megakaryocytic Essential thrombocythemia Fibroblast Myelofibrosis with myeloid metaplasia

6 NEOPLASTIC BONE MARROW DISORDERS Features common to all myeloproliferative disorders are: Occur gradually Occur in the middle aged or elderly Clinically the patients present with anemia or polycythemia, leukocytosis and thrombocytosis with bizarre, abnormally functional platlets Hepatosplenomegaly Hypercellular bone marrow that may become fibrotic May terminate in an acute leukemia

7 NEOPLASTIC BONE MARROW DISORDERS Polycythemia – polycythemia is a general term used to describe an erythrocytosis with increased RBCs and hemoglobin. Need to distinguish between relative (due to decreased plasma volume) and absolute Is classified into 3 groups Relative – caused by dehydration, hemoconcentration Primary polycythemia (polycythemia vera) Secondary polycythemia Both the primary and secondary forms result in an absolute increase in the number of RBCs

8 HEMACRIT IN POLYCYTHEMIA

9 POLYCYTHEMIA VERA Polycythemia vera is a primary, unregulated increase in RBCs with no identifiable initiating cause. All cells are usually increased with RBCs increased the most. The RBCs mature and function normally with a normal life span. The increase in RBCs is independent of erythropoietin stimulation

10 POLYCYTHEMIA VERA Clinically: The increased RBCs result in a sluggish circulation and when the hematocrit exceeds 60% this leads to hypertension After 2-10 years the bone marrow may fail 5-10% terminate in an acute leukemia – this is exacerbated by treatment with myelosuppressive drugs Splenomegaly is common Lab findings Increased RBC count and hematocrit Normochromic, normocytic cells Normal to slightly increased reticulocyte count

11 POLYCYTHEMIA VERA 2/3 show a leukocytosis with a slight shift to the left Increased platlets that are giant and functionally abnormal The arterial oxygen saturation is normal Treatment Phlebotomy Myelosuppressive drugs

12 POLYCYTHEMIA VERA

13 SECONDARY POLYCYTHEMIA There are three groups of causes of secondary polycythemia: Secondary to tissue hypoxia Arterial oxygen saturation is low The increase in RBCs is due to a normal physiologic increase in erythropoietin Causes include Living at high altitudes Chronic pulmonary disease

14 SECONDARY POLYCYTHEMIA Familial and associated with a high affinity oxygen or a 2,3 DPG deficiency Arterial oxygen saturation is normal Partial pressure at which hemoglobin is 50% saturated is decreased The increase in RBCs is due to a normal physiologic increase in erythropoietin Secondary to tumors Arterial oxygen saturation is normal Partial pressure at which hemoglobin is 50% saturated is normal The increase in RBCs is due to an inappropriate increase in erythropoietin

15 LAB DIFFERENTIATION OF POLYCYTHEMIA

16 ESSENTIAL THROMBOCYTHEMIA In essential thrombocythemia the megakaryocytic cell line is most prominently affected Clinical and lab findings include: Occurs most often in those that are or years-old. Most common feature is bleeding and thrombosis Platlet count is x 10 9 /L and the platlets are giant, bizarre and functionally abnormal. They tend to aggregate leading to clogged capillaries and the production of tear drop RBCs Anemia may occur due to bleeding, but 1/3 have a slight erythrocytosis Leukocytosis with a shift to the left occasionally occurs

17 ESSENTIAL THROMBOCYTHEMIA Prognosis/therapy Platlet pheresis (remove the platlets) Anticoagulants to prevent thrombosis Radiation or chemotherapy

18 ESSENTIAL THROMBOCYTHEMIA

19 MYELOFIBROSIS WITH MYELOID METAPLASIA Myelofibrosis with myeloid metaplasia (extramedullary hematopoiesis) – there are three features associated with this disease: Unchecked proliferation of hematopoietic elements Progressive bone marrow fibrosis Extramedullary hematopoiesis Clinical findings: This is a chronic, gradual disease occurring in individuals over 50 years of age Most symptoms come from anemia or pressure from the enlarged spleen Hematopoiesis may occur in the kidneys, adrenal glands, and lymph nodes as well as in the spleen and and liver Osteosclerosis ( increased bone density) is common

20 MYELOFIBROSIS WITH MYELOID METAPLASIA Lab findings: A moderate leukoerythroblastic anemia with striking anisocytosis and poikilocytosis. Many tear drop cells, which are formed as the RBCs try to squeeze into the circulation, are found. Leukocytosis with a shift to the left Giant, bizarre platlets The bone marrow may be difficult to aspirate A bone marrow biopsy reveals a hypercellular bone marrow with diffuse fibrosis and increased megakaryocytes

21 MYELOFIBROSIS WITH MYELOID METAPLASIA

22

23

24 DIFFERENTIATION OF MYELOPROLIFERATIVE DISORDERS

25 MYELODYSPLASTIC DISORDERS Myelodysplastic disorders are primary pluripotential stem cell disorders characterized by: One or more peripheral blood cytopenias resulting from ineffective hematopoiesis Prominent maturation abnormalities in the bone marrow (dyspoiesis or dysplasia) Usually occurs in the elderly Commonly progress to an acute leukemia Are refractory to treatment

26 MYELODYSPLASTIC DISORDERS Clinically: Patients usually present with weakness due to anemia that is refractory to treatment Patients may have chronic infections due to neutropenia Patients may have hemorrhaging due to thrombocytopenia Laboratory findings: Cytopenias and dysplasia are seen on the peripheral smear Abnormal maturation of RBCs leads to oval macrocytes Abnormal maturation of WBCs leads to hyposegmented, hypogranulated neutrophils Abnormal maturation of platlets leads to giant platlets with abnormal granulation

27 MYELODYSPLASTIC SYNDROME Note oval macrocytes:

28 MYELODYSPLASTIC SYNDROME Note hyposegmented, hypogranulated neutrophil:

29 MYELODYSPLASTIC SYNDROME Note large platlets

30 MYELODYSPLASTIC SYNDROME Note micromegakaryocyte:

31 MYELODYSPLASTIC SYNDROME Note large, agranular platlet:

32 MYELODYSPLASTIC SYNDROME Myelodysplastic syndromes are classically classified into five subgroups: Refractory anemia (RA) Refractory anemia with ringed sideroblasts (RARS) Refractory anemia with excess blasts (RAEB) Chronic myelomonocytic leukemia (CMML) Refractory anemia with excess blasts in transformation (RAEBt) A new classification system has been developed, but will not be discussed here.

33 DIFFERENTIATION OF MDS

34 SURVIVAL AND LEUKEMIC PROGRESSION OF MDS SUBGROUPS

35 OVERLAP OF MDS WITH OTHER HEMATOLOGIC DISORDERS


Download ppt "NEOPLASTIC DISORDERS OF THE BONE MARROW Those that are not leukemias."

Similar presentations


Ads by Google