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HEMOBLASTOSES. ANEMIAS. assistant of professor Nechiporenko G.V.

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Presentation on theme: "HEMOBLASTOSES. ANEMIAS. assistant of professor Nechiporenko G.V."— Presentation transcript:

1 HEMOBLASTOSES. ANEMIAS. assistant of professor Nechiporenko G.V.

2 HEMOBLASTOSES HEMOBLASTOSES are tumoral diseases of hemo-lymphopoietic tissue. HEMOBLASTOSES are tumoral diseases of hemo-lymphopoietic tissue. They are subdivided into: They are subdivided into: 1) Leukemias 2) Peripheral lymphomas

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5 Acute lymphoblastic leukemia (ALL) Lymphoblasts are very immature cells with large nuclei that contain nucleoli. ALL is more common in children than adults. Many cases of ALL in children respond well to treatment, and many are curable.

6 Necrotic tonsillitis in acute leukemia Necrotic tonsillitis in acute leukemia

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9 Chronic lymphocytic leukemia (CLL) Mature lymphocytes are increased markedly in number. A disease is most often seen in older adults;it responds poorly to treatment, but it is indolent.

10 Lymph nodes in chronic lympholeukemia

11 Liver in chronic lympholeukemia

12 Bone marrow of a patient with acute myeloblastic leukemia. There are many large immature myelocytes and one megakaryocyte at the right center

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15 Blood smear, initial presentation of chronic myeloid leukemia (CML) - High power

16 Blood smears, chronic myelogenous leukemia (CML) blast phase (left)

17 Bone marrow in chronic myeloleukemia

18 Spleen in chronic myeloleukemia

19 Liver in chronic myeloleukemia

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22 The skull demonstrates the characteristic rounded "punched out" lesions of multiple myeloma.

23 Round lesions filled with a soft reddish material are indicative of foci of myeloma in this section of vertebral bone.

24 There are numerous plasma cells with eccentric nuclei and a perinuclear halo of clearer cytoplasm in a smear of bone marrow aspirate from a patient with multiple myeloma.

25 Cell with paraprotein in multiple myeloma

26 Hodgkin's Disease - Classification Type Histologic Features FrequencyPrognosis Nodular sclerosis Bands of fibrosis, lacunar cells Most frequent type, more common in women Good, most are stage I or II Mixed cellularity Composed of many different cells Most frequent in older persons, second most frequent overall Fair, most are stage III Lymphocyte predominance Mostly B-cells and few Reed- Sternberg variant cells Uncommon Good, most are stage I or II Lymphocyte depletion Many Reed- Sternberg cells and variants Uncommon Poor, most are stage III or IV

27 Hodgkin's Disease - Staging StageCharacteristics IOnly a single lymph node site or extranodal site is involved IITwo or more lymph node sites on one side of the diaphragm are involved, or limited contiguous extranodal site involvement IIILymph node sites on both sides of the diaphragm are involved, with splenic or limited contiguous extradodal site involvement, or both IVExtensive involvement of extranodal sites, with or without lymph node involvement

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29 Here is a 5 cm lymph node (obviously from a patient with lymphadenopathy). The node should normally be soft and pink and less than 1 cm in size. This lymph node is involved with Hodgkin's disease.

30 This is a liver that is involved with Hodgkin's disease. The staging of Hodgkin's disease is very important in determining therapy.

31 Spleen in Hodgkin’s disease Spleen in Hodgkin’s disease

32 Mixed Hodgkin’s disease Mixed cellularity type of Hodgkin’s disease

33 Hodgkin's disease, nodular sclerosis type. Note the bands of pink collagenous tissue dividing the field in this lymph node.

34 These are the lacunar cells characteristic for the nodular sclerosis type of Hodgkin's disease.

35 This is a high power view of a Reed-Sternberg cell seen with Hodgkin's disease. Note the large, prominent nucleoli.

36 Non-Hodgkin's Lymphomas Type Histologic Features Immunogenetics Clinical Features Small Lymphocytic Lymphoma Small and well- differentiated B lymphocytes, with diffuse effacement of nodal architecture and no follicles CD19, 5; Bcl-2 and Bcl-6 expression Seen in older adults, it is essentially the solid tissue (lymph nodal) component of chronic lymphocytic leukemia; disease tends to be generalized but with indolent course and prolonged survival; some may transform to more aggressive lymphomas

37 Follicle Center Lymphoma (predominantly small cell) Nodal architecture is effaced by monotonous, crowded follicles composed of monomorphous small cleaved B- lymphocytes CD19, 20, 79a; t(14:18); Bcl-2 expression Most common type, seen in adults, often involves multiple lymph nodes, course is indolent, with prolonged survival, though some may transform to a large cell lymphoma

38 Diffuse Large B- cell Lymphoma Cells are large, with prominent nucleoli and abundant cytoplasm and many mitoses. Most are B-cell, but 20% are T-cell phenotype CD19, 20, 79a; some have t(14;18); some have Bcl-2 and Bcl- 6 expression; linked to EBV infection; negative TdT Though often localized, they tend to be aggressive extranodal masses; seen in adults and children, can be seen in HIV infection Burkitt Lymphoma Intermediate sized B-lymphocytes (small-noncleaved cells) CD10, 19, 20, 79a; t(8:14) is characteristic; African form linked to EBV infection; negative TdT Endemic in Africa with mandibular and abdominal involvement; sporadic elsewhere with abdominal involvement; affects mainly children and young adults

39 High-grade B-cell Lymphoma (small non-cleaved) Burkitt-like Lymphoma Intermediate sized B- lymphocytes (small non- cleaved cells) CD19, 20 Sporadic; may be seen with HIV infection Precursor T or B- cell Lymphoblastic Lymphoma/Leuke mia (Lymphoblastic Lymphoma) Intermediate sized lymphocytes in a diffuse pattern B-cells are CD19, 20, sometimes CD10; T-cells are CD3 and 8; all are TdT positive Seen in children and adolescents; T-cell type often in mediastinum; very aggressive and can progress to acute lymphocytic leukemia Mantle Cell Lymphoma Small to medium sized B cells CD 19, 20, 43; t(11;14); Bcl-1 (Cyclin D1) expression Seen in adults in middle age; often advanced at diagnosis and may be extranodal, including multifocal submucosal nodules in bowel

40 Marginal Zone Lymphoma Small to medium sized B cells CD19, 20, 79a; negative CD5 and 10 Seen in middle aged adults; typically arises in areas of immune activation (Hashimoto thyroiditis, Sjogren syndrome, H. pylori gastritis); similar lesions asociated with mucosal lymphoid tissue are called MALTomas (mucosa- associated lymphoid tissue tumors); may transform to diffuse large B-cell lymphoma

41 Lymph node, follicular lymphoma, low grade

42 This is a malignant lymphoma, small cleaved cell type, follicular (also known as: malignant lymphoma, poorly differentiated lymphocytic type, nodular). This is a malignant lymphoma, small cleaved cell type, follicular (also known as: malignant lymphoma, poorly differentiated lymphocytic type, nodular). Here is a lymph node involved by lymphoma. The capsule of the node has been invaded and the lymphomatous cells extend into the surrounding adipose tissue. Note that the follicles are numerous and irregularly sized. Here is a lymph node involved by lymphoma. The capsule of the node has been invaded and the lymphomatous cells extend into the surrounding adipose tissue. Note that the follicles are numerous and irregularly sized.

43 Small bowel, Burkitt lymphoma

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45 Malignant lymphoma is typically extranodal in AIDS. Seen here in small intestine are two mass lesions on the mucosal surface.

46 Extranodal malignant lymphoma in AIDS is often multifocal. Seen here in liver are two mass lesions on the cut surface. The color can range from white to tan to red, often intermixed.

47 Non-Hodgkin's lymphomas seen in the central nervous system with AIDS are essentially clonal expansions of Epstein-Barr virus infected lymphocytes. These lymphomas are high grade (immunoblastic) and agressive, with a poor prognosis.

48 Anemia Anemia is a reduction in the concentration of the hemoglobin in the blood. It is usually accompanied by reduction in the number of red blood cells (with the exception of iron-deficiency types, thalassemia). Poikilocytosis (different size), anisocytosis (different shape) of erythrocytes can develop in blood. Erythroblasts, normoblasts, megaloblasts appear also. Anemia is a reduction in the concentration of the hemoglobin in the blood. It is usually accompanied by reduction in the number of red blood cells (with the exception of iron-deficiency types, thalassemia). Poikilocytosis (different size), anisocytosis (different shape) of erythrocytes can develop in blood. Erythroblasts, normoblasts, megaloblasts appear also.

49 Classification of Anemias. A.PATHOPHYSIOLOGIC I. Anemia due to increased blood loss Acute post-haemorrhagic anemia Acute post-haemorrhagic anemia Chronic blood loss Chronic blood loss II. Anemias due to increased red cell destruction (Hemolytic anemias) A.Extrinsic (extracorpuscular) red cell abnormalities B.Intrinsic (intracorpuscular) red cell abnormalities

50 III. Anemias due to impaired red cell production a)Cytoplasmic maturation defects a)Cytoplasmic maturation defects Deficient haem synthesis: Iron deficiency anemia Deficient haem synthesis: Iron deficiency anemia Deficient globin synthesis: Thalassaemic syndromes Deficient globin synthesis: Thalassaemic syndromes b)Nuclear maturation defects b)Nuclear maturation defects Vitamin B12 and/or folic acid deficiency: Megaloblastic anaemia Vitamin B12 and/or folic acid deficiency: Megaloblastic anaemia c)Defect in stem cell proliferation and differentiation c)Defect in stem cell proliferation and differentiation Aplastic anemia Aplastic anemia Pure red cell aplasia Pure red cell aplasia Anemia of chronic disorders Anemia of chronic disorders Bone marrow infiltration Bone marrow infiltration Congenital anemia Congenital anemia

51 B.MORPHOLOGIC I.Microcytic, hypochromic II. Normocytic, normochromic III. Macrocvtic. normochromic

52 о Ischemia of kidney in acute posthemorrhagic anemia

53 Extramedullar hemopoiesis in liver with chronic posthemorrhagic anemia

54 Pernicious anemia (B12 or/and folic acid deficiency) Cause is absence of gastromucoprotein secretion by parietal cells due to heredital dysfunction of fundal glands in stomach. Autoimmune processes take place: autoantibodies blockade gastromucoprotein and vit. B 12, so erythropoiesis changes in megaloblastic type. Hemolysis predominates over hemopoiesis. Cause is absence of gastromucoprotein secretion by parietal cells due to heredital dysfunction of fundal glands in stomach. Autoimmune processes take place: autoantibodies blockade gastromucoprotein and vit. B 12, so erythropoiesis changes in megaloblastic type. Hemolysis predominates over hemopoiesis.

55 Macro-ovalocytes in a case of Pernicious Anemia.

56 Aplastic anemia Hematopoietic elements in this bone marrow biopsy are markedly reduced.

57 Hemosiderosis of Spleen Hemosiderosis of Spleen

58 Hemolytic anemia. Hemoglobinuric nephrosis Hemoglobinuric nephrosis


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