Extracorpuscular anemias immunity related mechanical trauma infections
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 2 nd or 3 rd gravidity (faster IgG response).
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
Infections malaria - 4 subtypes of plasmodia, Asia, Africa - Anopheles (mosquito) Plasmodium falciparum - malignant tertian malaria
Anemias of diminished erythropoiesis lack of –iron –folic acid –vitamin B 12 –less frequently - pyridoxin, thiamin
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
Folic acid and vitamin B 12 (Cobalamin) deficiency anemia (megaloblastic) folic acid deficiency - gravidity, severe alcoholics, drug abusers. Sometimes celiakia, malabsorbtion. B 12 - like folic acid deficiency, in addition - peripheral nerves and spinal chord demyelinization
Aplastic anemia pancytopenia, erythrocytopenia, agranulocytosis, thrombocytopenia in half number of cases - idiopatic sometimes after irradiation, myelotoxic drugs
ALL (acute lymphoblastic leukemia) 80 % of childhood leukemias 5 - 10 % Philadelphia chromosome (22 9) prognosis - relatively good, 90 % - remission, if translocation worse prognosis
AML (acute myeloid leukemia) adult middle age prognosis bad, 5-year survival - 10 - 15 % hiatus leukemicus bone marrow transplantation
Chronic myeloproliferative disorders CML CLL polycytemia vera HCL myeloid metaplasia with myelofibrosis essential thrombocytemia
CML (chronic myeloid leukemia) adults middle or younger age “pyoid” bone marrow Philadelphia chromosome - 90 % patients bad prognosis
CLL (chronic lymphatic leukemia) older age long asymptomatic period, non- characteristic symptoms course and prognosis variable related to malignant lymphomas
Polycytemia vera proliferation of erythroid, myeloid and megakaryocytic line increased blood viscosity, blood volume borne marrow highly cellular hypertension, thromboses, bleeding
Hairy cell leukemia chronic B-cell line leukemia fine cytoplasmic projections - immunohistochemistry, phase contrast, EM hepatomegaly, splenomegaly therapy - purine analogues
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
Leukemias - general morphology bone marrow soft - “pyoid” (CML) splenomegaly - CML lymph nodes enlargement - CLL hepatomegaly - CML, CLL sometimes other organs infiltrated
Non-neoplastic white cells disordes leukopenia - neutropenia (agranulocytosis) –impaired granulopoiesis - bone marrow failure –destruction of granulocytes - immunity, drugs reactive leukocytosis - infections infectious mononucleosis - EBV
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.
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