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Approach to the adult with unexplained pancytopenia

Authors
Nancy Berliner, MD
John M Gansner, MD, PhD
Section Editor
Stanley L Schrier, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Pancytopenia refers to decreases in all peripheral blood lineages and is considered to be present when all three cell lines are below the normal reference range.

Pancytopenia can be associated with a multitude of disease states, some of which are life threatening. A thoughtful history and physical examination along with focused laboratory studies are required to establish a diagnosis and select proper management. In most cases of pancytopenia, referral to a hematologist will be important for purposes of diagnosis and/or management.  

The diagnostic approach to an adult patient with pancytopenia will be discussed here. The evaluation of isolated neutropenia, anemia, and thrombocytopenia are presented separately. (See "Approach to the adult with unexplained neutropenia" and "Approach to the adult patient with anemia" and "Approach to the adult with unexplained thrombocytopenia".)

DEFINITION OF PANCYTOPENIA

Pancytopenia refers to decreases in all peripheral blood lineages. Many disorders that cause pancytopenia can also cause bicytopenia (ie, decreases in only two cell lines); thus, in most cases the evaluation of bicytopenia is similar to that presented here.

Individual laboratories typically establish their own reference ranges for hemoglobin/hematocrit, white blood cell count, and platelet count. These institutional cutoff values supersede published reference standards such as those published by the World Health Organization [1]:

                         
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Literature review current through: Nov 2017. | This topic last updated: Mar 30, 2017.
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References
Top
  1. Valent P. Low blood counts: immune mediated, idiopathic, or myelodysplasia. Hematology Am Soc Hematol Educ Program 2012; 2012:485.
  2. Young NS, Abkowitz JL, Luzzatto L. New Insights into the Pathophysiology of Acquired Cytopenias. Hematology Am Soc Hematol Educ Program 2000; :18.
  3. Pascutti MF, Erkelens MN, Nolte MA. Impact of Viral Infections on Hematopoiesis: From Beneficial to Detrimental Effects on Bone Marrow Output. Front Immunol 2016; 7:364.
  4. Marks PW. Hematologic manifestations of liver disease. Semin Hematol 2013; 50:216.
  5. Risitano AM, Maciejewski JP, Selleri C, Rotoli B. Function and malfunction of hematopoietic stem cells in primary bone marrow failure syndromes. Curr Stem Cell Res Ther 2007; 2:39.
  6. Jain A, Naniwadekar M. An etiological reappraisal of pancytopenia - largest series reported to date from a single tertiary care teaching hospital. BMC Hematol 2013; 13:10.
  7. Weinzierl EP, Arber DA. Bone marrow evaluation in new-onset pancytopenia. Hum Pathol 2013; 44:1154.
  8. Shimamura A, Alter BP. Pathophysiology and management of inherited bone marrow failure syndromes. Blood Rev 2010; 24:101.
  9. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med 2014; 371:654.
  10. Devitt KA, Lunde JH, Lewis MR. New onset pancytopenia in adults: a review of underlying pathologies and their associated clinical and laboratory findings. Leuk Lymphoma 2014; 55:1099.
  11. Sohn SK, Suh JS, Lee J, Lee KB. Pancytopenic prodrome (pre-ALL) of acute lymphoblastic leukemia in adults: possible pathogenesis. Korean J Intern Med 1998; 13:64.
  12. Bhargava R, Kar R, Mahapatra M, Saxena R. The clinico-hematological profile of hairy cell leukaemia: a single centre experience. Hematology 2010; 15:162.
  13. Galani KS, Subramanian PG, Gadage VS, et al. Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital. Indian J Pathol Microbiol 2012; 55:61.
  14. Somasundaram V, Purohit A, Aggarwal M, et al. Hairy cell leukemia: A decade long experience of North Indian Hematology Center. Indian J Med Paediatr Oncol 2014; 35:271.
  15. Gabreyes AA, Abbasi HN, Forbes KP, et al. Hypocupremia associated cytopenia and myelopathy: a national retrospective review. Eur J Haematol 2013; 90:1.
  16. Sabel AL, Gaudiani JL, Statland B, Mehler PS. Hematological abnormalities in severe anorexia nervosa. Ann Hematol 2013; 92:605.
  17. Levy RN, Sawitsky A, Florman AL, Rubin E. Fatal aplastic anemia after hepatitis. Report of five cases. N Engl J Med 1965; 273:1118.
  18. Zeldis JB, Dienstag JL, Gale RP. Aplastic anemia and non-A, non-B hepatitis. Am J Med 1983; 74:64.
  19. Young NS, Calado RT, Scheinberg P. Current concepts in the pathophysiology and treatment of aplastic anemia. Blood 2006; 108:2509.
  20. Zimran A, Kay A, Gelbart T, et al. Gaucher disease. Clinical, laboratory, radiologic, and genetic features of 53 patients. Medicine (Baltimore) 1992; 71:337.
  21. Preis M, Lowrey CH. Laboratory tests for paroxysmal nocturnal hemoglobinuria. Am J Hematol 2014; 89:339.
  22. Mir N, Costello C, Luckit J, Lindley R. HIV-disease and bone marrow changes: a study of 60 cases. Eur J Haematol 1989; 42:339.
  23. Kyeyune R, Saathoff E, Ezeamama AE, et al. Prevalence and correlates of cytopenias in HIV-infected adults initiating highly active antiretroviral therapy in Uganda. BMC Infect Dis 2014; 14:496.
  24. Ehehalt U, Schunk M, Jensenius M, et al. Leishmaniasis acquired by travellers to endemic regions in Europe: a EuroTravNet multi-centre study. Travel Med Infect Dis 2014; 12:167.
  25. Bosilkovski M, Krteva L, Dimzova M, et al. Human brucellosis in Macedonia - 10 years of clinical experience in endemic region. Croat Med J 2010; 51:327.
  26. et al, Gonen I, Sozen H, Kayal O. Brucellosis: Evaluation of 201 cases in an endemic area of Mediterranean Basin. Acta Medica Mediterranea 2014; 30:121.
  27. Achi HV, Ahui BJ, Anon JC, et al. [Pancytopenia: a severe complication of miliary tuberculosis]. Rev Mal Respir 2013; 30:33.
  28. Maartens G, Willcox PA, Benatar SR. Miliary tuberculosis: rapid diagnosis, hematologic abnormalities, and outcome in 109 treated adults. Am J Med 1990; 89:291.
  29. Wolfromm A, Rodriguez C, Michel M, et al. Spectrum of adult Parvovirus B19 infection according to the underlying predisposing condition and proposals for clinical practice. Br J Haematol 2015; 170:192.
  30. Bareau B, Rey J, Hamidou M, et al. Analysis of a French cohort of patients with large granular lymphocyte leukemia: a report on 229 cases. Haematologica 2010; 95:1534.
  31. Parikh SA, Kapoor P, Letendre L, et al. Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Mayo Clin Proc 2014; 89:484.
  32. Otrock ZK, Eby CS. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol 2015; 90:220.
  33. Li F, Yang Y, Jin F, et al. Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China. Orphanet J Rare Dis 2015; 10:20.
  34. Schram AM, Campigotto F, Mullally A, et al. Marked hyperferritinemia does not predict for HLH in the adult population. Blood 2015; 125:1548.
  35. Clines GA. Mechanisms and treatment of hypercalcemia of malignancy. Curr Opin Endocrinol Diabetes Obes 2011; 18:339.
  36. Wilson FP, Berns JS. Tumor lysis syndrome: new challenges and recent advances. Adv Chronic Kidney Dis 2014; 21:18.
  37. Sutton JF, Stacey M, Kearns WG, et al. Increased risk for aplastic anemia and myelodysplastic syndrome in individuals lacking glutathione S-transferase genes. Pediatr Blood Cancer 2004; 42:122.