Pancytopenia

Pancytopenia

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  PANCYTOPENIA ASWATHY .A ASSISTANT PROFESSOR DEPARTMENTOF PATHOLOGY
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  PANCYTOPENIA Pancytopenia is amedical condition in which there is reduction in the number of red blood cell, platelet as well as WBC.  If only two parameters from the full blood count are low the term BICYTOPENIA.  It is commonly seen in India  It is the combination of anemia,leukopenia, and thrombocytopenia :- Hb < 13.5 in males & 11.5 in females Leucocyte count < 4x109 /l Platelet count < 150x109 /l
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  CAUSES 1) Decreased bonemarrow function 2) Increased peripheral destruction DECREASED BONE MARROW FUNCTION A) Aplastic anemia -Idiopathic Secondary Inherited B)Myelodysplastic syndrome C)Bone marrow infiltration- Leukemia, lymphoma , multiple myeloma, tumors , hairy cell leukemia etc.. D)Nutritional deficiency - Vit B12 and folic acid E)PNH F)Myelofibrosis G) Hemophagocytic syndrome
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  INCREASED PERIPHERAL DESTRUCTION Hypersplenism  Over whelming infection  Miscellaneous
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  APPROACH OF PANCYTOPENIA •History • Clinical Examination • CBC • Peripheral smear examination • Bone Marrow Aspiration • Bone Marrow Biopsy • Other specific investigations
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  A) HISTORY • Mildpancytopenia is often symptomless and detected incidentally when a full blood count/CBC is performed for another reason. • Duration of symptoms • Transfusions ,Haemoglobinuria • Dietary history • Socio economic status • Exposure to – Drugs ( anti cancer, Antibiotic, Anti epileptics, Anti thyroid (Aplastic) Barbiturates, Phenytoin, OCP ( B12 & FA) • Chemicals (Aplastic) • Radiation (Aplastic) • Infections (Aplastic) - TB, Malaria • Weight loss, fever – malignancy & inflammatory • Jaundice – Hep B & C • Joint Pain – SLE • Blood Loss
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  B) CLINICAL EXAMINATION •Eye examination- Retinal hemorrhage (thrombocytopenia),Leukemic infiltrates (acute leukemia) Aundiced sclera (paroxysmal nocturnal hemoglobinuria, hepatitis, cirrhosis) Epiphora (dyskeratosis congenita) • Oral examination -Oral petechiae or hemorrhage (thrombocytopenia) Stomatitis or cheilitis (neutropenia, vitamin B12 deficiency) Gingival hyperplasia (leukemia) Oral candidiasis or pharyngeal exudate (neutropenia, herpes family virus infections) • Cardiovascular examination Tachycardia, edema, congestive cardiac failure (all signs of symptomatic anemia) Evidence of prior cardiac surgery (cardiac disease associated with congenital syndromes)
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  • Respiratory examination Clubbing(lung cancer) Tachypnea (sign of symptomatic anemia) • Abdominal examination Right upper quadrant tenderness (hepatitis) Lymphadenopathy (infection, lymphoproliferative disorder, HIV disease) • Chronic liver disease -Splenomegaly (infection, myeloproliferative and lymphoproliferative disorders) • Skin examination- Malar rash (SLE) ,Purpura/bruising (thrombocytopenia) • Reticular pigmentation, dysplastic nails (dyskeratosis congenita) ,Hypopigmented areas. Hyperpigmentation, café au lait (Fanconi anemia) • Musculoskeletal examination- Short stature (Fanconi anemia, other congenital syndromes),Swelling/synovitis (SLE), Abnormal thumbs (e.g., Fanconi anemia) • Signs associated with HIV disease- Morbilliform rash early . Kaposi sarcoma, ulcerating nodules later
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  • Pancytopenia +Back pain + Hypercalcemia -(multiple myeloma) • Pancytopenia + Malar rash or GN = SLE • Pancytopenia + HSM = Leukemia • Pancytopenia + Absent thumb +Child
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  C) CBC RBC, HB,PLATELET,CT – Decreased BT,ESR- Increased D) PERIPHERAL SMEAR EXAMINATION Red Cell Morphology  Normocytic normochromic with no anisopoikilocytosis , few macrocytes, no nRBCs ,  Reticulocytopenia – Aplastic anemia  Macro ovalocytes with Howell Jolly Bodies – Megaloblastic anemias  Tear drop cells, HJ bodies & Basophilic stippling – MDS  nRBCs, Sickle cells – Aplastic crisis in Hemolytic anemia. WBCs  Leucopenia (mostly mature ~80%) – Aplastic anaemia  Neutrophils present in increased number with toxic granules, shift to left – Infections  Basophilic stippling, hyper segmented neutrophils – Megaloblastic anaemia  Blasts – subleukemic leukemia  Hypo granular neutrophils, pseudo Pelger Heut Anomaly – MDS
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  Platelets • Normal count– rules out aplastic anemia • Giant platelets – MDS/ Hypersplenism E) BONE MARROW EXAMINATION • Examination of bone marrow is almost always indicated in cases of • pancytopenia unless the cause is otherwise apparent (e.g., established liver disease with portal hypertension). • The bone marrow exam consists of both an aspirate and a trephine biopsy, • The differential diagnosis of pancytopenia may be broadly classified based on the bone marrow cellularity (reduced cellularity indicates decreased production of blood cells, whereas normal/increased cellularity indicates ineffective production or increased destruction or sequestration of blood cells). • Specifically, bone marrow aspirate permits examination of: • Cytology (megaloblastic change, dysplastic changes, abnormal cell infiltrates, hemophagocytosis , and infection [e.g., Leishman-Donovan bodies])
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  • Immunophenotyping (acuteand chronic leukemias, lympho proliferative disorders) • Cytogenetics (myelodysplasia, acute and chronic leukemias, lymphoproliferative disorders). Aspiration • Empty particles, markedly hypocellular, only scattered mature • lymphocytes & sometimes excess plasma cells – aplastic anemias • Pockets of cellularity with widespread hypocellularity – evolving aplastic anemia • Hypocellular with BM Blasts (>20%) – hypoplastic leukemia • Hypocellular BM with dysplastic megakaryocytes – hypoplastic MDS Scattered proerythroblasts with large nuclear inclusions inhypocellular BM – Parvovirus.
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  Biopsy • Erythroid hyperplasiawith megablastosis – megaloblastic anemia • Trilineage dysplasis with ringed sideroblasts on pearl’s stain – MDS • Infiltration by RS Cells – HL • Infiltration with malignant cells – metastasis In PNH & Fanconi’s anemia – early stage will show hypercellular normal appearing marrow F) OTHER TESTS  Vit B 12 and folic acid assay  Fibrin and D-Dimer , PT,APTT- Increased  ALP and ACP - Increased  Urine BJP  SLE  Chest X -ray and Bone X- ray
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  TREATMENT Treatment for anaemia, thrombocytopenia and leucopenia