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Hematopoietic and lymphoid systems

Hematopoietic and lymphoid systems. main entities - disorders exam questions and very concise text - www.lfhk.cuni.cz/patanat. Hematopoietic and lymphoid systems - exam questions. Posthemorrhagic and hemolytic anemias Anemias of diminished erythropoiesis; pernicious anemia Polycythemia

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Hematopoietic and lymphoid systems

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  1. Hematopoietic and lymphoid systems • main entities - disorders • exam questions and very concise text - www.lfhk.cuni.cz/patanat

  2. Hematopoietic and lymphoid systems - exam questions • Posthemorrhagic and hemolytic anemias • Anemias of diminished erythropoiesis; pernicious anemia • Polycythemia • Bleeding disorders • DIC • Lymph node pathology - review • Spleen pathology - review • Thymus pathology - review • Non-Hodgkin’s lymphomas • Hodgkin’s disease • Acute leukemias • Chronic leukemias • Myeloproliferative disorders • Plasma cell dyscrasias • Histiocytoses • Storage diseases

  3. Anemia - causes • posthemorrhagic • hemolytic • impaired red cell production

  4. Blood loss - posthemorrhagic anemias • acute - hypovolemia, shock, rapid hemodilution, slowly increasing hematopoesis (sufficient amount of iron) • chronic - GIT, female genital tract - iron loss (increased hematopoesis)

  5. Hemolytic anemias - general features • increased rate of RBCs destruction • increased amount of iron • hypercellular bone marrow • reticulocytes in peripheral blood

  6. Hemolytic anemias - RBCs destruction • intracorpuscular (intrinsic) - hereditary, acquired are rare • extracorpuscular (extrinsic) - acquired, immunity, mechanical trauma, infections

  7. Intracorpuscular anemias • hereditary • acquired

  8. Hereditary • spherocytosis • sickle cell anemia • thalassemia - T. maior, T. minor • glucose-6-phosphate dehydrogenase deficiency

  9. Acquired • paroxysmal nocturnal hemoglobinuria

  10. Extracorpuscular anemias • immunity related • mechanical trauma • infections

  11. Immunity related • autoimmune anemias • Erythroblastosis fetalis (hemolytic disease of the newborn) - related mainly to Rh system (D- antigen), less to AB0 systém. Passage of fetal RBCs through the placenta during last trimester (no cytotrophoblast) or during childbirth. Mother antibodies cross the placenta. • Concurent AB0 incompatibility protects the mother against Rh immunization - RBCs removed from maternal circulation. The blood dose - 1 ml. IgG response - cross the placenta, IgM - does not cross, the response faster in 2nd or 3rd gravidity (faster IgG response).

  12. Mechanical trauma • prostethic valves - more in metallic or plastic prostheses than in bioprostheses • microangiopathic hemolytic anemia - squeezed RBCs in narrowed vessels - DIC, SLE, malignant hypertension

  13. Infections • malaria - 4 subtypes of plasmodia, Asia, Africa - Anopheles (mosquito) • Plasmodium falciparum - malignant tertian malaria

  14. Anemias of diminished erythropoiesis • lack of • iron • folic acid • vitamin B12 • less frequently - pyridoxin, thiamin

  15. Iron deficiency anemia (sideropenic) • lack of iron in the food - veggies; malabsorption - sprue • increased demand - gravidity • chronic loss - GIT, menstrual bleeding • morphology - microcytosis, low RBCs volume, pallor, spoon-shaped nails

  16. Folic acid and vitamin B12 (Cobalamin) deficiency anemia (megaloblastic) • folic acid deficiency - gravidity, severe alcoholics, drug abusers. Sometimes celiakia, malabsorbtion. • B12 - like folic acid deficiency, in addition - peripheral nerves and spinal chord demyelinization

  17. Aplastic anemia • pancytopenia, erythrocytopenia, agranulocytosis, thrombocytopenia • in half number of cases - idiopatic • sometimes after irradiation, myelotoxic drugs

  18. Myelophtisis • bone marrow metastases - breast, lungs, prostate • multiple myeloma, TBC

  19. Bleeding disorders - hemorrhagic diatheses • vascular fragility - vitamin C deficiency - scurvy • DIC, thrombocytopenia, coagulopathies

  20. DIC • Blood clotting - starts from soft tissue (tissue thromboplastin) or endothelial damage (factor XII.) • Causes: 1/ release of clotting factors - amniotic fluid embolization, cytoplasmic granules - promyelocytic leukemia, mucus - Ca, Gram-negative sepsis 2/ extensive endothelial damage, burns, SLE

  21. DIC - morphology • fibrin microthrombi within capillaries - kindeys, brain, heart, lungs, adrenal gland (Waterhouse - Friderichsen sy), hypophysis (Sheehan sy) • acute DIC - bleeding, chronic - thrombotic

  22. Thrombocytopenia • Idiopathic thrombocytopenic purpura (ITP) • Thrombotic thrombocytopenic purpura (TTP) (m. Moschowitz) - microthrombi composed of platelets

  23. Coagulation disorders • acquired - prothrombin, f. VII., IX., and X. - hepatic damage • hereditary - • hemophilia A • hemophilia B

  24. Leukemias • leukemic form • aleukemic form • leukemic infiltration of the liver, spleen, lymph nodes • primary site - bone marrow

  25. Leukemias - classification • maturation: • acute leukemias • chronic myeloproliferative disorders • cell types: • lymphatic • myeloid

  26. Acute leukemias • clinical course • sudden onset • anemia, fever, infections, hemorrhagic diathesis • bone pain (bone marrow expansion) • generalized lymphadenopathy (ALL), splenomegaly, hepatomegaly • CNS symptoms - headache, vomitus, paralysis

  27. ALL (acute lymphoblastic leukemia) • 80 % of childhood leukemias • 5 - 10 % Philadelphia chromosome (22  9) • prognosis - relatively good, 90 % - remission, if translocation worse prognosis

  28. AML (acute myeloid leukemia) • adult middle age • prognosis bad, 5-year survival - 10 - 15 % • hiatus leukemicus • bone marrow transplantation

  29. Chronic myeloproliferative disorders • CML • CLL • polycytemia vera • HCL • myeloid metaplasia with myelofibrosis • essential thrombocytemia

  30. CML (chronic myeloid leukemia) • adults middle or younger age • “pyoid” bone marrow • Philadelphia chromosome - 90 % patients • bad prognosis

  31. CLL (chronic lymphatic leukemia) • older age • long asymptomatic period, non-characteristic symptoms • course and prognosis variable • related to malignant lymphomas

  32. Polycytemia vera • proliferation of erythroid, myeloid and megakaryocytic line • increased blood viscosity, blood volume • borne marrow highly cellular • hypertension, thromboses, bleeding

  33. Hairy cell leukemia • chronic B-cell line leukemia • fine cytoplasmic projections - immunohistochemistry, phase contrast, EM • hepatomegaly, splenomegaly • therapy - purine analogues

  34. Myeloid metaplasia with myelofibrosis • bone marrow fibrotic, hypocellular • neoplastic stem cells within the spleen • unknown ethiology of bone marrow fibrosis • splenomegaly, trilinear hematopoiesis in the spleen, prominent megakaryocytes

  35. Leukemias - general morphology • bone marrow soft - “pyoid” (CML) • splenomegaly - CML • lymph nodes enlargement - CLL • hepatomegaly - CML, CLL • sometimes other organs infiltrated

  36. Non-neoplastic white cells disordes • leukopenia - neutropenia (agranulocytosis) • impaired granulopoiesis - bone marrow failure • destruction of granulocytes - immunity, drugs • reactive leukocytosis - infections • infectious mononucleosis - EBV

  37. Plasma cell dyscrasias • multiple myeloma • localized plasmacytoma • Waldenström’s macroglobulinemia • heavy-chain disease • primary amyloidosis • monoclonal gammopathy of undetermined significance

  38. Multiple myeloma morphology • osteolytic lesions - prominent mainly in the scull • histologically - diffuse infiltration by neoplastic plasma cells • kidney - myeloma nephrosis - neoplastic plasma cells, casts within distal tubules

  39. Histiocytoses X • acute disseminated Langerhans’ cell histiocytosis (Letterer - Siwe) • unifocal and multifocal - eosinophilic granuloma • multifocal histiocytosis (Hand- Schüller-Christian disease)

  40. Malignant lymphomas • non-Hodgkin’s lymphomas • Hodgkin’s lymphoma

  41. Non-Hodgkin’s lymphomas • nodular • diffuse • Working Formulation, Kiel Classification, REAL, WHO

  42. Malignant lymphomas - some entities • ML of CLL type • follicular centre ML • diffuse large cell ML • Burkitt’s lymphoma

  43. Extranodal ML • mycosis fungoides • Sézary’s syndrome • MALToma

  44. Hodgkin’s ML • nodular lymphocyte predominance Hodgkin’s lymphoma • classical Hodgkin’s lymphoma • lymphocyte rich • nodular sclerosis • mixed cellularity • lymphocytic depletion

  45. Hodgkin’s ML - staging • I. Single lymph node region or single extralympatic organ. • II. Two or more lymph node regions or limited contiguous extralymphatic organ on the same side of diaphragm. • III. Two or more lymph node regions or limited contiguous extralymphatic organ on both sides of diaphragm. • IV. Multiple, disseminated foci of involvement, both sides of diaphragm + bone marrow.

  46. Lymph nodes -non-neoplastic diseases • reactive lymphadenitis • acute non-specific • chronic non-specific • speciphic lymphadenitis • TBC (BCG) • Cat Scratch Disease

  47. Spleen - overview • enlargement • massive • moderate • mild • rupture • necrosis

  48. Thymus - overview • thymic hyperplasia • tumors • thymoma • benign thymoma - 90 % • thymic carcinoma

  49. Thymomas • epithelial predominance • lymphocyte predominance • mixed • appendant disease - myasthenia gravis

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