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PRESENTED BY-DR.BISWAJEETA SAHA(1ST YR PGT)
 MODERATOR-DR.S.RAMAN,KIMS,BHUBANESWAR
DEFINITION

 Combination of anemia, leucopenia and thrombocytopenia

 Hb<9g/dl

 TLC<4000/cmm

 TPC<1.5 lacs/cmm
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
Fanconis 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
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 neutrophilsmegaloblastic anemia
 If blasts presentsubleukemic leukemia
 Hypogranular neutrophils,pseudo pelger heut anomaly--MDS


 PLATELETS-
 Normal TPC rules out aplastic anemia
 Giant plateletsMDS /hypersplenism
History taking
 Duration of 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)
 Weight loss , fever (malignancy, inflammatory)
 Jaundice (Hep B & C)
 Evidence of bleeding
 Infections
    TB, Malaria
 Joint pains
    SLE
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
 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
Bone marrow examination
Almost almost 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 hypocellularityevolving
   phase of aplastic anemia
 Hypocellular BM with Blasts(>20%)----hypoplastic leukemia
 Hypocellular BM with Dysplastic megakaryocyteshypoplastic MDS
 Scattered proerythroblasts with large nuclear inclusions in hypocellular BM
   parvo virus
In hypercellular BM


  Erythroid hyperplasia with megablastosismegaloblastic anemia

  Trilineage dysplasia with ringed sideroblasts on perl stainMDS

  Infiltration by RS cellsHL

  Infiltration with malignant cellsmetastasis

  In PNH and fanconis anemiaearly stage will show hypercellular normal
    appearing marrow
Specific testing pinpoints diagnosis 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
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
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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Investigations of pancytopenia

  • 1. PRESENTED BY-DR.BISWAJEETA SAHA(1ST YR PGT) MODERATOR-DR.S.RAMAN,KIMS,BHUBANESWAR
  • 2. DEFINITION Combination of anemia, leucopenia and thrombocytopenia Hb<9g/dl TLC<4000/cmm TPC<1.5 lacs/cmm
  • 3. 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 Fanconis 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
  • 4. 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 neutrophilsmegaloblastic anemia If blasts presentsubleukemic leukemia Hypogranular neutrophils,pseudo pelger heut anomaly--MDS PLATELETS- Normal TPC rules out aplastic anemia Giant plateletsMDS /hypersplenism
  • 5. History taking Duration of 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)
  • 6. Weight loss , fever (malignancy, inflammatory) Jaundice (Hep B & C) Evidence of bleeding Infections TB, Malaria Joint pains SLE
  • 7. 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
  • 8. 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
  • 9. Bone marrow examination Almost almost 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 hypocellularityevolving phase of aplastic anemia Hypocellular BM with Blasts(>20%)----hypoplastic leukemia Hypocellular BM with Dysplastic megakaryocyteshypoplastic MDS Scattered proerythroblasts with large nuclear inclusions in hypocellular BM parvo virus
  • 10. In hypercellular BM Erythroid hyperplasia with megablastosismegaloblastic anemia Trilineage dysplasia with ringed sideroblasts on perl stainMDS Infiltration by RS cellsHL Infiltration with malignant cellsmetastasis In PNH and fanconis anemiaearly stage will show hypercellular normal appearing marrow
  • 11. Specific testing pinpoints diagnosis 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
  • 12. 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
  • 13. 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