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Diminished Erythropoiesis, Most common nutritional disorder in world -common in toddlers, teenage girls,women of childbearing age -females w/ less total body Fe and less storage Functional and Storage Fe compartments: -Functional-80% is in Hgb-rest in myoglobin, catalase, cytochromes -Storage-hemosiderin, ferritin-20% of total boday Fe -ferritin esp in liver, spleen, bone marrow, sk. mm. -in liver parenchymal cells, in macs elsewhere -ferritin in lysosomes aggregates into hemosiderin granules -little in circulation, tells adequacy of Fe stores -Transferrin-made in liver, 33% saturable in NL-deliver to cells Morphology Periph - Hypochromic, Microcytic RBCs -Hgb only in narrow periph rim Bone Marrow-inc. EPO activity->inc. normoblasts Hgb, Hct dec, some poikilocytosis Transferrin saturation% Low serum Fe, inc. Fe-binding capacity/transferrin receptors, Anemias of Diminished Erythropoiesis Anemia of Chronic Dz Most common anemia cause among hospitalized pts -can mimic Fe-def anemia -reduced RBC prolif, impaired Fe utilization Causes: 1. Chronic Microbial Infx-osteomyelitis, bac. endocarditis, lung abscess 2. Chronic Immune Dzs-RA, regional enteritis 3. Neoplasms-Hodgkin Dz, lung, breast CAs Low serum Fe, REDUCED toal Fe-binding cap -lots of Fe stored in mononuc phagos-defect in recycling Mainly caused by marrow HYPOproliferation b/c low EPO response IL-1, TNF-a, IFN-g->reduced renal EPO generation RBCs NL or microcytic, hypochromic RULE OUT Fe-DEF ANEMIA VIA:inc. storage Fe in macs, high ferritin, reduced total Fe-binding capacity, transferrin receptor in NL range Tx underlying condition or give rEPO, Anemias of Diminished Erythropoiesis Megaloblastic Anemias -impaired DNA synth -defective nuclear maturation Pernicious Anemia-Vit B12 deficiency -due to atrophic gastritis->fail to make IF -parietal cells make IF, binds in duodenum -IF receptors in ileum, x-port via transcobalamin (from stomach to duod, B12 bound to R-binder) -no B12->"trap" F5-CH3-FH4 ->administer Folate->improve anemia, not neuro -no B12->methylmalonyl CoA buildup (protein cata.) ->ABNL lipids into neurons->myelin breakdown, Anemias of Diminished Erythropoiesis Aplastic Anemia Pancytopenia (RBCs, neutropenia, plts) Most "known-etiology" cases due to exposure to chemicals/drugs -Myeloxins-benzene, chloramphenicol, alkylators, antimetabolites -Drugs-phenylbutazone, methylphenylethylhydantoin, streptomycin, chlorpromazine -Whole-body irradiation-dose related -Viral hepatitis, HIV -Fanconi Anemia-defective DNA repair, w/ congenital anomalies -65% IDIOPATHIC Morphology -hypocellular bone marrow (DRY TAP) -drugs destroy other organs, like fatty liver/kidneys -systemic hemosiderosis w/ multiple transfusions in some pts -NO SPLENOMEGALY, Langerhans Cell Histiocytosis -clonal prolifs of APC-DCs w/ HLA-DR, CD1a =histiocytosis X=3 conditions (Letterer-Siwe, Hand-Schuller Christian dzs, eosinophilic granuloma) CHARACTERISTIC HX BODIES in DCs (Burbeck granules) -tennis-racket appearance under EM), -also, detect CD1a for Dx 3 Presentations: -all can have spontaneous regression or CTX Hand-Schuller-Christian Triad Hand-Schuller-Christian triad= Calvarial Defects + Diabetes Insipidus + Exophthalmos, Anemias of Diminished Erythropoiesis Megaloblastic Anemias -impaired DNA synth -defective nuclear maturation Folate Deficiency Anemia -same anemia as B12 def, but no neuro -FH4 is middleman that transfers 1-Carbon unit: 1. Purine synth 2. homocysteine->Met (req B12) 3. deoxythymidylate monophosphate synth ->REQUIRED FOR DNA SYNTH Can get 1-C unit from FIGlu -lack FH4->excess FIGlu, low thymine, Langerhans Cell Histiocytosis -clonal prolifs of APC-DCs w/ HLA-DR, CD1a =histiocytosis X=3 conditions (Letterer-Siwe, Hand-Schuller Christian dzs, eosinophilic granuloma) CHARACTERISTIC HX BODIES in DCs (Burbeck granules) -tennis-racket appearance under EM), -also, detect CD1a for Dx 3 Presentations: -all can have spontaneous regression or CTX Letterer-Siwe Dz Letterer-Siwe dz ɚ yo, maybe adults -seborrheic-type lesion on trunk, scalp -due to Langerhans infiltration -w/ hepatosplenomegaly, LNs, pulmonary lesions, ->osteolytic bone lesions -into marrow->anemia, thrombocytopenia, infx -fatal w/out Tx, 50% survival w/ CTX, Anemias of Diminished Erythropoiesis Megaloblastic Anemias -impaired DNA synth -defective nuclear maturation In Peripheral Blood ->LARGE precursors MCV ->anisocytotic, normochromic, can lack pallor -can look hyperchromic b/c large -ret. count low, some nucleated RBCs -large PMNs, 6+ nuclear lobules, Langerhans Cell Histiocytosis -clonal prolifs of APC-DCs w/ HLA-DR, CD1a =histiocytosis X=3 conditions (Letterer-Siwe, Hand-Schuller Christian dzs, eosinophilic granuloma) CHARACTERISTIC HX BODIES in DCs (Burbeck granules) -tennis-racket appearance under EM), -also, detect CD1a for Dx 3 Presentations: -all can have spontaneous regression or CTX Eosinophilic Granuloma AKA Unifocal/Multifocal Langerhans Cell Histiocytosis -expanding, erosive accum of Langs in marrow spaces -w/ eos, lymphos, plasma cells, neutrophils -eos scattered, mature or sheetlike -esp in skull, ribs, femur-also in skin, lungs, stomach UNIFOCAL-usually skeletal-(-)Sx or pain/tenderness -can heal spontaneously, or cure w/ excisions/irrad. MULTIFOCAL-children-fever, diffuse eruptions of scalp, ear canals -frequent OM, mastoiditis, URIs -maybe mild hepatosplenomegaly, LNs -50% w/ post. pituitary stalk involvement->DIABETES INSIPIDUS, Anemias of Diminished Erythropoiesis Pure Red Cell Aplasia Only erythropoiesis impaired, not grans, plts -Can be primary-from autoimmunity->immunosupps help -Can be 2ndary to neoplasm (thymoma, leukemia)