Pancytopenia resident survival guide
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sanjana Nethagani, M.B.B.S.[2]
Synonyms and keywords: Approach to pancytopenia, Approach to anemia
Pancytopenia Resident Survival Guide Microchapters |
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Overview |
Causes |
Diagnosis |
Treatment |
Do's |
Don'ts |
Overview
Pancytopenia is described as a decrease in the 3 cell lines which are red blood cells, white blood cells and platelets. Clinically, pancytopenia is defined as hemoglobin< 9gm, white blood cell count< 4,000/cmm and platelets < 100,000/cmm. It can be due to decreased production in the bone marrow or increased destruction of cells in the periphery. Pancytopenia can also be caused due to drugs such as chemotherapy agents. Treatment involves identifying the underlying cause and appropriate therapy.
Causes
Decreased production of cells
Increased peripheral destruction
Other causes
- Chemotherapy
- Parvovirus B-19 infection
- Autoimmune conditions
- Human immunodeficiency virus infection
- Tuberculosis
- Lymphoma
For a full list of pancytopenia causes, click here.
Diagnosis
Diagnosis of pancytopenia is outlined in the algorithm below.
Thorough history must be taken including ❑ Symptoms of autoimmune disease such as * joint pain and swelling *rash * lymphadenopathy etc ❑ History of malignancy ❑ History of recent infections ❑ History of usage of drugs which cause marrow suppression such as * azathioprine and other chemotherapy drugs * corticosteroids * linezolid * chloramphenicol etc ❑ History of chemo or radiotherapy ❑ nutritional status ❑ Family history of anemia or pancytopenia | |||||||||||||||||||||||||||||||||||||||||
Manifestations of decrease in each cell line Decrease in RBCs ❑ dyspnea ❑ fatigue ❑ pallor ❑ chest pain Decrease in WBCs ❑ increased susceptibility to infections ❑ fever Decrease in platelets ❑ petechiae ❑ easy bruising ❑ bleeding Other signs to look for are ❑ Signs of liver disease ❑ Splenomegaly ❑ Lymphadenopathy ❑ Signs of eating disorders ❑ Signs of alcoholism ❑ Signs of Vitamin B12 or folate deficiency | |||||||||||||||||||||||||||||||||||||||||
First line investigations ❑ Peripheral smear Look for dysplastic cells such as macrocytes and blasts ❑ Complete blood count with reticulocyte count ❑ Iron studies ❑ Erythrocyte sedimentation rate ❑ C reactive protein ❑ Liver function tests ❑ Lactate dehydrogenase level | |||||||||||||||||||||||||||||||||||||||||
Additional investigations ❑ HIV serology ❑ Hepatitis serology ❑ Screening for tuberculosis ❑ Antinuclear antibody level ❑ Coomb's test | |||||||||||||||||||||||||||||||||||||||||
Bone marrow aspiration | |||||||||||||||||||||||||||||||||||||||||
Hypocellular marrow | Cellular marrow | ||||||||||||||||||||||||||||||||||||||||
Morphology | |||||||||||||||||||||||||||||||||||||||||
Investigate for following conditions ❑ Aplastic anemia ❑ Congenital aplastic anemia such as Diamond blackfan syndrome ❑ Blast cell morphology and CD cell markers ❑ Paroxysmal nocturnal hemoglobinuria | Normal morphology | Dysplastic cells, marrow fibrosis or infiltration | |||||||||||||||||||||||||||||||||||||||
Systemic causes | |||||||||||||||||||||||||||||||||||||||||
Infections such as ❑ HIV serology ❑ Hepatitis serology ❑ PCR for tuberculosis ❑ Smear for malarial parasite ❑ LD body for leishmaniasis | ❑ Megaloblastic anemia ❑ Vitamin assays ❑ antiparietal cell antibodies ❑ evaluation for malabsorption syndromes | ||||||||||||||||||||||||||||||||||||||||
Treatment
Treatment of pancytopenia is outlined in the algorithm below.
Abbreviations: LDH- Lactate dehydrogenase, RBC- Red blood cells, G-CSF - Granulocyte colony stimulating factor, CNS- Central nervous system
Pancytopenia diagnosis established ❑ Hemoglobin < 10gm/dl ❑ Leucocyte count < 4000/mcL ❑ Platelet count < 150,000/mcL | |||||||||||||||||||||||||||||||||||||
Initial investigations ❑ Peripheral blood smear ❑ Coagulation profile ❑ Serum LDH level ❑ Complete blood count with reticulocyte level ❑ Bilirubin level ❑ Coomb's test | |||||||||||||||||||||||||||||||||||||
Acute bleeding Prompt treatment with ❑ Insert two large bore IVs ❑ Type and match blood ❑ IV fluids to correct hypovolemia ❑ Packed RBCs ❑ Fresh frozen plasma ❑ Platelet transfusion for platelet count < 10,000/mcL to prevent intracranial bleeding ❑ Monitor vital signs and laboratory parameters at frequent intervals | Neutropenic fever ❑ Send two sets of blood cultures ❑ Empirical intravenous antibiotic therapy should be started with an antipseudomonal beta lactam such as cefepime/ a carbepenem or piperacillin-tazobactam ❑ Modify antibiotics once culture and sensitivity results come back ❑ If MRSA is suspected, vancomycin/daptomycin or linezolid may be started ❑ Empirical treatment with antifungals is not recommended ❑ Periodic assessment of response to therapy ❑ Monitor vitals regularly ❑ G-CSF therapy is recommended in patients undergoing chemotherapy | ||||||||||||||||||||||||||||||||||||
Hypocellular marrow Investigate for and treat probable causes such as ❑ Aplastic anemia * ATG * Drugs such as cyclophosphamide * Blood transfusions * Bone marrow transplant is curative ❑ Paroxysmal nocturnal hemoglobinuria * Warfarin to decrease risk of thrombosis * Blood transfusions when needed * Eculizumab therapy * Meningococcal vaccination ❑ Congenital aplastic anemia * Hematopoietic stem cell transplant | Dysplastic cells in bone marrow or infiltration ❑ Leukemia * Induction and consolidation chemotherapy * CNS prophylaxis * Bone marrow transplant ❑ Lymphoma * Chemotherapy * rituximab * stem cell transplantation ❑ Myelodysplasia * Supportive care with blood products and erythropoietin * Eligible candidates may receive bone marrow transplantation * Chemotherapy | Nutritional causes ❑ | D04 | D05 | |||||||||||||||||||||||||||||||||
Do's
- Screen for infections like hepatitis, HIV, Parvovirus-B19, Epstein Barr virus, etc.
- Periodic, regular blood tests must be done for patients on methotrexate or other marrow suppressing drugs.
Dont's
- Don't prescribe Aspirin or NSAIDs, or any other drugs that may precipitate bleeding in patients with pancytopenia.
- Don't wait for culture and sensitivity reports in case of neutropenic fever or sepsis in pancytopenic patients. Prompt treatment with broad spectrum antibiotics is key.
- Don't progress to treatment without correcting underlying nutritional deficiencies such as Vitamin B12 or folate deficiency.