Investigations of pancytopenia

Investigations of pancytopenia

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  PRESENTED BY-DR.BISWAJEETA SAHA(1STYR PGT) MODERATOR-DR.S.RAMAN,KIMS,BHUBANESWAR
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  DEFINITION  Combination ofanemia, leucopenia and thrombocytopenia  Hb<9g/dl  TLC<4000/cmm  TPC<1.5 lacs/cmm
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  ETIOLOGY Hypocellular marrow Cellular marrow with Primary marrow disease def/syst diseases with cellular marrow •Aplastic anemia •Vit B12 and folic acid •Aleukemic leukemia •Hypoplastic MDS deficiency •Hairy cell leukemia •Post chemotherapy •Hypersplenism •MDS •Fanconi’s anemia •Kala azar •Marrow necrosis •Diamond-Shwachman •SLE,sjogrens •Myelofibrosis syndrome syndrome,sarcoidosis •PNH •Transfusion associated •Tuberculosis,brucelosis •HPS GVHD •Metastatic solid tumors •Aplastic crisis in •Alcoholism hemolytic anemia •Storage diseases •Drugs •Infections
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  Peripheral smear  RBC- Normocytic normochromic with few macrocytes,no anisopoikilocytosis,no nRBCs,reticulocytopenia----aplastic anemia  Macroovalocytes,howel jolly bodies-megaloblastic anemia  Tear drop cells,howel jolly bodies,basophilic stippling--MDS  nRBCs,sickle cells-aplastic crisis in sickle cell anemia  WBC-  Leucopenia,mostly mature lymphocytyes(80-90%)---aplastic anemia  Neutrophils present in increased number,with toxic granules,shift to left— infections  Basophilic stippling,hypersegmented neutrophils—megaloblastic anemia  If blasts present—subleukemic leukemia  Hypogranular neutrophils,pseudo pelger heut anomaly--MDS  PLATELETS-  Normal TPC rules out aplastic anemia  Giant platelets—MDS /hypersplenism
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  History taking  Durationof symptoms  How many blood transfusions,intervals between transfusions  h/o hemoglobinuria  Dietary history  Socio economic status  Exposure to  Drugs, especially antineoplastic, antibiotics (chloramphenicol, sulphonamides),anti epileptics and antithyroid drugs (Aplastic)  barbiturates, phenytoin, and oral contraceptives (B12)  Exposure to radiation (Aplastic)  Chemical exposure  Benzene & insecticides (Aplastic)  Infections  Viruses (viral hepatitis,EBV, parvovirus, and HIV. (Aplastic)
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   Weight loss, fever (malignancy, inflammatory)  Jaundice (Hep B & C)  Evidence of bleeding  Infections  TB, Malaria  Joint pains  SLE
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  General physical examination Eye examination  Retinal hemorhage  Leukemic infiltration  Jaundiced sclera(PNH,hepatitis,cirrhosis)  Epiphora(dyskeratosis congenita)  Oral examination  Oral petechaie  Stomatitis/chelitis  Gingivial hyperplasia  Oral candidiasis
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   Cardiovascular examination  Musculoskelatal system  Tachycardia,edema,CCF Short stature Synovitis  Respiratory system Abnormal thumb  Clubbing  Tachypnoea  Abdominal examination  Hepatomegaly  Lymphadenopathy  Splenomegaly  Skin examination  Malar rash  Purpura  Reticular pigmentation,dysplastic nails(dyskeratosis congenita)  Hypopigmented areas  hyperpigmentation
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  Bone marrow examination Almostalmost always indicated in cases of pancytopenia unless the cause is apparent.  Both aspiration and biopsy required  Sample for cytogenetics and immunophenotyping,culture and serological studies IN THE ASPIRATE:  Empty particles,markedly hypocellular,only scattered mature lymphocytes and sometimes plasma cells in excess---aplastic anemia  Sometimes pockets of cellularity with widespread hypocellularity—evolving phase of aplastic anemia  Hypocellular BM with Blasts(>20%)----hypoplastic leukemia  Hypocellular BM with Dysplastic megakaryocytes—hypoplastic MDS  Scattered proerythroblasts with large nuclear inclusions in hypocellular BM— parvo virus
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  In hypercellular BM  Erythroid hyperplasia with megablastosis—megaloblastic anemia  Trilineage dysplasia with ringed sideroblasts on perl stain—MDS  Infiltration by RS cells—HL  Infiltration with malignant cells—metastasis  In PNH and fanconis anemia—early stage will show hypercellular normal appearing marrow
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  Specific testing pinpointsdiagnosis in following conditions  Fanconis anemia: diepoxybutane (DEB) test for chromosomal breakage in peripheral blood lymphocyte  Lymphoproliferative disorders:immunophenotyping,cytogenetics,lymph node biopsy  PNH:peripheral blood immunophenotyping for deficiency of phosphatidylinositol glycan linked molecules on peripheral blood cells(cd 16,cd 55,cd 59),HAMs test  CMV:serum IgM,IgG  EBV:serum monospot,viral capsid antigen(VCA),Epstein barr nuclear antibody(EBNA)  Leishmaniasis:blood and bone marrow culture,ELISA  Serum PSA:prostatic ca
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  pancytopenia History,examination,CBC,retic count,serum iron/TIBC Blast,hypo Unexplained No specific Hypersegmented Palpable spleen Sepsis/bleeding PMNs,marked with increased retic granular splenomegal finding PMNs y/M anisopoikilocytosis protein/meta stasis Trial of B12 and folic acid No response response Acid hemolysis test Hypersplenism/m alaria/leishmanias is HIV antibody Bone marrow aspiration and biopsy
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  CBC AND PS •LFT •PNH and IBMFS •Bone marrow •B12/Folate levels investigations biopsy and •Coagulation aspirate profile •Viral serology •Autoimmune profile Bonemarrow Blood/bm cytogenetics immunophen otyping
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