Pancytopenia is a reduction in the number of RBC, WBC and platelet. It's a combination of anaemia, leukopenia and thrombocytopenia. Pancytopenia caused by Decreased bone marrow function and increased peripheral destruction. diseases are diagnosed by physical examination, complete blood counting, peripheral smear examination, bone marrow examination and other special methods. Treatment to pancytopenia is treated to anaemia, thrombocytopenia and leukopenia
2. PANCYTOPENIA
Pancytopenia is a medical condition in which there is reduction in the number of red blood
cell, platelet as well as WBC.
ď If only two parameters from the full blood count are low the term BICYTOPENIA.
ď It is commonly seen in India
ď 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
3. CAUSES
1) Decreased bone marrow 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
5. APPROACH OF PANCYTOPENIA
⢠History
⢠Clinical Examination
⢠CBC
⢠Peripheral smear examination
⢠Bone Marrow Aspiration
⢠Bone Marrow Biopsy
⢠Other specific investigations
6. A) HISTORY
⢠Mild pancytopenia 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 â 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 â malignancy & inflammatory
⢠Jaundice â Hep B & C
⢠Joint Pain â SLE
⢠Blood Loss
7. 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)
8. ⢠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, cafÊ 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
9. ⢠Pancytopenia + Back pain + Hypercalcemia -(multiple myeloma)
⢠Pancytopenia + Malar rash or GN = SLE
⢠Pancytopenia + HSM = Leukemia
⢠Pancytopenia + Absent thumb +Child
10. C) CBC
RBC, HB, PLATELET,CT â Decreased
BT,ESR- Increased
D) PERIPHERAL SMEAR EXAMINATION
Red Cell Morphology
ď Normocytic normochromic with no anisopoikilocytosis , few macrocytes, no nRBCs ,
ď Reticulocytopenia â Aplastic anemia
ď Macro ovalocytes with Howell Jolly Bodies â Megaloblastic anemias
ď Tear drop cells, HJ bodies & Basophilic stippling â MDS
ď nRBCs, Sickle cells â Aplastic crisis in Hemolytic anemia.
WBCs
ď Leucopenia (mostly mature ~80%) â Aplastic anaemia
ď Neutrophils present in increased number with toxic granules, shift to left â Infections
ď Basophilic stippling, hyper segmented neutrophils â Megaloblastic anaemia
ď Blasts â subleukemic leukemia
ď Hypo granular neutrophils, pseudo Pelger Heut Anomaly â MDS
11.
12. Platelets
⢠Normal count â rules out aplastic anemia
⢠Giant platelets â 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])
13. ⢠Immunophenotyping (acute and 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 â aplastic anemias
⢠Pockets of cellularity with widespread hypocellularity â evolving aplastic anemia
⢠Hypocellular with BM Blasts (>20%) â hypoplastic leukemia
⢠Hypocellular BM with dysplastic megakaryocytes â hypoplastic MDS Scattered proerythroblasts with large
nuclear inclusions inhypocellular BM â Parvovirus.
14. Biopsy
⢠Erythroid hyperplasia with megablastosis â megaloblastic anemia
⢠Trilineage dysplasis with ringed sideroblasts on pearlâs stain â MDS
⢠Infiltration by RS Cells â HL
⢠Infiltration with malignant cells â metastasis In PNH & Fanconiâs anemia â early stage will show hypercellular normal
appearing marrow
F) OTHER TESTS
ď Vit B 12 and folic acid assay
ď Fibrin and D-Dimer , PT,APTT- Increased
ď ALP and ACP - Increased
ď Urine BJP
ď SLE
ď Chest X -ray and Bone X- ray