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==Historical Perspective==
==Historical Perspective==
The history of pancytopenia relates to the history of each of its individual entities, namely anemia, thrombocytopenia, and leukopenia. Pancytopenia was not recognized as a distinct clinical entity until after each of its other subcomponents were characterized.


==Classification==
==Classification==


==Pathophysiology==
==Pathophysiology==
The pathophysiology of pancytopenia relates to the underlying etiology. In most cases, pancytopenia is due to a disruption in trilineage hematopoiesis. This means that the bone marrow is not appropriately producing erythrocytes, leukocytes, and thrombocytocytes. The cause of the disruption in trilineage hematopoiesis is in turn due to the underlying cause of pancytopenia. For example, viral-mediated pancytopenia is caused by viral particles infecting hematopoietic cells and preventing normal cell division. Leukemia-mediated pancytopenia is typically due to marrow replacement of normal hematopoietic precursors, a process known as myelopthisis. Leukemic infiltration of the bone marrow creates a "crowding-out" phenomenon.


==Causes==
==Causes==

Revision as of 04:35, 13 March 2017

Pancytopenia is not equivalent with bone marrow suppression. Pancytopenia is a lab finding that may related to either bone marrow suppression or peripheral sequestration/destruction. For details about bone marrow suppression click here.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2] Ogheneochuko Ajari, MB.BS, MS [3] Shyam Patel [4]

Overview

Pancytopenia is the reduction in numbers of all three bone marrow cell types (RBCs + WBCs + platelets). It is not a disease, but rather a lab finding that may related to bone marrow suppression caused by either insufficient production (aplastic anemia), inability of cells or mature (myelodysplasia), replacement of normal bone marrow with fibrosis (myelofibrosis) or peripheral sequestration that is not related to the bone marrow (e.g. splenomegaly or hypersplenism). or destruction (such as hemolytic anemia. HIV (human immunodeficiency virus) is itself a cause of pancytopenia. Chemotherapy is associated with pancytopenia due to drug-mediated bone marrow suppression. Pancytopenia usually requires a bone marrow biopsy in order to distinguish among different causes.

Historical Perspective

The history of pancytopenia relates to the history of each of its individual entities, namely anemia, thrombocytopenia, and leukopenia. Pancytopenia was not recognized as a distinct clinical entity until after each of its other subcomponents were characterized.

Classification

Pathophysiology

The pathophysiology of pancytopenia relates to the underlying etiology. In most cases, pancytopenia is due to a disruption in trilineage hematopoiesis. This means that the bone marrow is not appropriately producing erythrocytes, leukocytes, and thrombocytocytes. The cause of the disruption in trilineage hematopoiesis is in turn due to the underlying cause of pancytopenia. For example, viral-mediated pancytopenia is caused by viral particles infecting hematopoietic cells and preventing normal cell division. Leukemia-mediated pancytopenia is typically due to marrow replacement of normal hematopoietic precursors, a process known as myelopthisis. Leukemic infiltration of the bone marrow creates a "crowding-out" phenomenon.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Causes by Organ System

Cardiovascular No underlying causes
Chemical/Poisoning Arsenic poisoning, arsenicals, benzene solvents, genotoxic therapy, glue vapors, gold, mutagen exposure, mutagen-detoxification (GSTq1-null), radium chloride, toxins, valproic acid poisoning
Dental No underlying causes
Dermatologic Chediak-Higashi disease, dyskeratosis congenita, eosinophilic fasciitis, melanoma, reticular dysgenesis, reticulosis, systemic lupus erythematosus, xeroderma pigmentosum
Drug Side Effect Acetaminophen and Oxycodone, aclarubicin, albendazole, alemtuzumab, alkylating antineoplastic agent, auranofin, azathioprine, aztreonam, boceprevir, busulfan, carbamazepine, carboplatin, cefadroxil, ceftazidime, certolizumab pegol, chemotherapy, chloramphenicol, chlorpromazine, chlorpropamide, cromolyn, cidofovir, clomipramine, cyclophosphamide, cytotoxic drugs, dactinomycin, docetaxel, doxorubicin, ethosuximide, febuxostat, flucytosine, gemcitabine, gemifloxacin mesylate, genotoxic therapy, idarubicin, Imipenem-Cilastatin,indomethacin , infliximab, interferon beta-1a, lincomycin hydrochloride, minocycline hydrochloride, non steroidal anti-inflammatory drugs, ofatumumab, omacetaxine, oxaprozin, oxcarbazepine, penicillamine, phenacemide , phenylbutazone, piperacillin, pralatrexate, propylthiouracil, pyrimethamine, rabeprazole, sulfonamides , tamoxifen, temozolomide, thiothixene, tolazamide, tolbutamide, topoisomerase II interactive agents, trifluoperazine, trimethadione, trimethoprim-sulfamethoxazole, valganciclovir hydrochloride
Ear Nose Throat Dubowitz syndrome, Jacobsen syndrome
Endocrine Osteoporosis, panhypopituitarism, thyroid carcinoma
Environmental No underlying causes
Gastroenterologic Banti's syndrome, cirrhosis, Gaucher's disease, hepatosplenic T-cell lymphoma, hypersplenism, intrinsic factor deficiency, malabsorption syndrome, Plummer-Vinson syndrome, portal hypertension, sarcoidosis, Schwachman-Diamond syndrome, splenomegaly
Genetic Albers-Schonberg disease, ataxia telangiectasia, Banti's syndrome, Bloom syndrome , cartilage-hair hypoplasia, Chediak-Higashi disease, Diamond-Blackfan anemia, DNA repair-deficiency disorder, Down syndrome, Dubowitz syndrome, dyskeratosis congenita, familial histiocytic reticulosis, familial monosomy 7, Fanconi anemia, Gaucher's disease, hemophagocytic lymphohistiocytosis, Hoyeraal-Hreidarsson syndrome, Jacobsen syndrome, intrinsic factor deficiency, neurofibromatosis 1, Niemann-Pick disease, osteopetrosis lethal, osteopetrosis, autosomal recessive 2, Pearson syndrome, Schwachman-Diamond syndrome, severe combined immunodeficiency, TAR syndrome, trisomy 8 mosaicism, Wiskott-Aldrich syndrome, xeroderma pigmentosum
Hematologic Acute lymphoblastic leukemia, acute myeloid leukemia, aggressive NK-cell leukemia, amegakaryocytic thrombocytopenia, aplastic anemia, autoimmune lymphoproliferative syndrome type 1, autoimmune lymphoproliferative syndrome type 2, bleeding (Excessive), bone marrow tumor, Castleman's disease, chronic lymphocytic leukaemia, chronic myeloid leukaemia, cyclical neutropenia, Diamond-Blackfan anemia, familial histiocytic reticulosis, familial myelofibrosis, Fanconi anemia, Gaucher's disease, hairy cell leukemia, hematopoietic stem cell transplantation, hemoglobin E disease, hemoglobin H disease, hemoglobin SC disease, hemophagocytic lymphohistiocytosis , hepatosplenic T-cell lymphoma, histiocytosis X, Hodgkin's lymphoma, hypersplenism, hypoplastic myelodysplastic syndromes, ineffective erythropoiesis, intestinal lymphangiectasia, intrinsic factor deficiency, iron deficiency, langerhans cell histiocytosis, leucoerythroblastic anemia, leukemia, lymphoma, malignant histiocytosis, megaloblastic anemia, myelodysplastic syndrome, myelofibrosis, myeloma, myelopathic anemia, non-Hodgkin lymphoma, paroxysmal nocturnal hemoglobinuria, Pearson syndrome, pernicious anemia, Plummer-Vinson syndrome, precursor B acute lymphoblastic leukemia, reticulosis, Schwachman-Diamond syndrome, sickle cell disease, TAR syndrome, t-cell large granular lymphocytic leukemia, Wiskott-Aldrich syndrome
Iatrogenic Radiation therapy, radioactive p32
Infectious Disease Babesiosis, brucellosis, dengue, Epstein-Barr virus , hepatitis, human immunodeficiency virus , infectious mononucleosis, kala-azar, Kikuchi disease, lassa fever, legionella pneumophila, mycobacterium tuberculosis, parvovirus B19 infection, Q fever, tuberculosis, viral infections, Whipple's disease
Musculoskeletal/Orthopedic Albers-Schonberg disease, ankylosing spondylitis, cartilage-hair hypoplasia, Ewing's sarcoma, osteoclastoma, osteopetrosis lethal, osteopetrosis, autosomal recessive 2, osteoporosis, osteosarcoma, TAR syndrome
Neurologic Ataxia telangiectasia, Down syndrome , neuroblastoma, neurofibromatosis 1, Niemann-Pick disease
Nutritional/Metabolic Anorexia nervosa, copper deficiency, folate deficiency, Gaucher's disease, glutathione synthase deficiency, iron deficiency, kwashiorkor, marasmus, obesity, vitamin B12 deficiency, vitamin C deficiency
Obstetric/Gynecologic Pregnancy
Oncologic Acute lymphoblastic leukemia, acute myeloid leukemia, aggressive NK-cell leukemia, bone marrow tumor, cancer, chondrosarcoma, chronic lymphocytic leukaemia, chronic myeloid leukaemia, epiphyseal tumors, Ewing's sarcoma, familial myelofibrosis, germ cell tumors , granuloma, hairy cell leukemia, hepatosplenic T-cell lymphoma, histiocytosis X, Hodgkin's lymphoma, hypoplastic myelodysplastic syndromes, langerhans cell histiocytosis, leukemia, lymphoma, melanoma, metastatic neoplasm, myelodysplastic syndrome, myelofibrosis, myeloma, neuroblastoma, non-Hodgkin lymphoma, osteoclastoma, osteosarcoma, precursor B acute lymphoblastic leukemia, prostate cancer, t-cell large granular lymphocytic leukemia, thymoma, thyroid carcinoma
Ophthalmologic No underlying causes
Overdose/Toxicity Colchicine toxicity
Psychiatric Anorexia nervosa
Pulmonary Sarcoidosis
Renal/Electrolyte Renal failure, chronic, systemic lupus erythematosus
Rheumatology/Immunology/Allergy Ankylosing spondylitis, auto-immune disorders, autoimmune lymphoproliferative syndrome type 1, autoimmune lymphoproliferative syndrome type 2, Castleman's disease, common variable hypogammaglobulinemia, common variable immune deficiency, eosinophilic fasciitis , Felty's syndrome, graft versus host disease, histiocytosis X, langerhans cell histiocytosis, rheumatoid disease, sarcoidosis, severe combined immunodeficiency, systemic lupus erythematosus, Wiskott-Aldrich syndrome
Sexual No underlying causes
Trauma No underlying causes
Urologic Prostate cancer
Miscellaneous Idiopathic, radioactive p32, tobacco use

Causes in Alphabetical Order

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3

Causes by Pathophysiology

Bone Marrow Failure

Peripheral Sequestration/Destruction

Causes by Mode of Inheritance

Congenital

Acquired

Differentiating [Disease] from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

The treatment of pancytopenia depends on the underlying cause. If pancytopenia is due to medication adverse effect, the offending agent should be discontinued.

Surgery

The is no role for surgery for pancytopenia. However, for immune thrombocytopenia purpura (ITP) and autoimmune hemolytic anemia (AIHA), splenectomy can be considered.

Prevention

References

  1. ↑ 1.0 1.1 1.2 Das Makheja K, Kumar Maheshwari B, Arain S, Kumar S, Kumari S (2013). "The common causes leading to pancytopenia in patients presenting to tertiary care hospital". Pak J Med Sci. 29 (5): 1108–11. PMC 3858928. PMID 24353701.
  2. ↑ Shah NR, Landi DB, Kreissman SG, Kulbachi E, Moran C (2011). "Presentation and outcomes for children with bone marrow necrosis and acute lymphoblastic leukemia: a literature review". J Pediatr Hematol Oncol. 33 (7): e316–9. doi:10.1097/MPH.0b013e318223fe9b. PMID 21941136.
  3. ↑ Guerrero A M, Lira V P, Bertin C P, Galleguillos V M, Ocqueteau T M (2005). "[Natural killer cell leukemia. Case report]". Rev Med Chil. 133 (4): 457–60. doi:/S0034-98872005000400010 Check |doi= value (help). PMID 15953954.
  4. ↑ Vande Zande VL, Mazza JJ, Yale SH (2004). "Hematologic and metabolic abnormalities in a patient with anorexia nervosa". WMJ. 103 (2): 38–40. PMID 15139557.
  5. ↑ Bacon BR, Treuhaft WH, Goodman AM (1981). "Azathioprine-induced pancytopenia. Occurrence in two patients with connective-tissue diseases". Arch Intern Med. 141 (2): 223–6. PMID 7458518.
  6. ↑ Poisnel E, Ebbo M, Berda-Haddad Y, Faucher B, Bernit E, Carcy B; et al. (2013). "Babesia microti: an unusual travel-related disease". BMC Infect Dis. 13: 99. doi:10.1186/1471-2334-13-99. PMC 3598249. PMID 23432953.
  7. ↑ Braier L (1983). "An hypothesis for the induction of leukemia by benzene". Arch Toxicol Suppl. 6: 42–6. PMID 6578748.
  8. ↑ Guler S, Kokoglu OF, Ucmak H, Gul M, Ozden S, Ozkan F (2014). "Human brucellosis in Turkey: different clinical presentations". J Infect Dev Ctries. 8 (5): 581–8. doi:10.3855/jidc.3510. PMID 24820461.
  9. ↑ Bajaj P, Clement J, Bayerl MG, Kalra N, Craig TJ, Ishmael FT (2014). "High-grade fever and pancytopenia in an adult patient with common variable immune deficiency". Allergy Asthma Proc. 35 (1): 78–82. doi:10.2500/aap.2014.35.3704. PMID 24433602.
  10. ↑ Abdel-Karim A, Frezzini C, Viggor S, Davidson LE, Thornhill MH, Yeoman CM (2009). "Dyskeratosis congenita: a case report". Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 108 (2): e20–4. doi:10.1016/j.tripleo.2009.03.042. PMID 19615640.
  11. ↑ Zhang X, Wang Z, Wang L, Yao H (2013). "An adult case of systemic Epstein-Barr virus-positive T/natural killer-cell lymphoproliferative disorder with good outcome". Int J Clin Exp Pathol. 6 (11): 2620–4. PMC 3816837. PMID 24228130.
  12. ↑ Roche C, Roche NC, Thefenne H, Saidi R, De Pina JJ, Molinier S; et al. (2011). "[Pancytopenia and folate deficiency: a case report]". Ann Biol Clin (Paris). 69 (3): 331–5. doi:10.1684/abc.2011.0584. PMID 21659050.
  13. ↑ Suyama T, Obara N, Kawai K, Yamada K, Kusakabe M, Kurita N; et al. (2013). "[Acute myeloid leukemia possibly originating from the same clone of testicular germ cell tumor]". Rinsho Ketsueki. 54 (8): 764–8. PMID 24005437.
  14. ↑ Agbaht K, Altintas ND, Topeli A, Gokoz O, Ozcebe O (2007). "Transfusion-associated graft-versus-host disease in immunocompetent patients: case series and review of the literature". Transfusion. 47 (8): 1405–11. doi:10.1111/j.1537-2995.2007.01282.x. PMID 17655584.
  15. ↑ Fino P, Fioramonti P, Onesti MG, Passaretti D, Scuderi N (2012). "Skin metastasis in patient with hairy cell leukemia: case report and review of literature". In Vivo. 26 (2): 311–4. PMID 22351675.
  16. ↑ Giri PP, Pal P, Ghosh A, Sinha R (2013). "Infection-associated haemophagocytic lymphohistiocytosis: a case series using steroids only protocol for management". Rheumatol Int. 33 (5): 1363–6. doi:10.1007/s00296-011-2291-2. PMID 22193223.
  17. ↑ Jain A, Naniwadekar M (2013). "An etiological reappraisal of pancytopenia - largest series reported to date from a single tertiary care teaching hospital". BMC Hematol. 13 (1): 10. doi:10.1186/2052-1839-13-10. PMC 4177001. PMID 24238033.
  18. ↑ Konoplev S, Medeiros LJ, Lennon PA, Prajapati S, Kanungo A, Lin P (2007). "Therapy may unmask hypoplastic myelodysplastic syndrome that mimics aplastic anemia". Cancer. 110 (7): 1520–6. doi:10.1002/cncr.22935. PMID 17701956.
  19. ↑ Mattina T, Perrotta CS, Grossfeld P (2009). "Jacobsen syndrome". Orphanet J Rare Dis. 4: 9. doi:10.1186/1750-1172-4-9. PMC 2670819. PMID 19267933.
  20. ↑ Trejo-Pérez JA, Miranda-Novales MG, Solórzano-Santos F, Cabrera-Muñoz L, Díaz-Ponce H (1993). "[Kala-azar in Mexico: report of 2 cases]". Bol Med Hosp Infant Mex. 50 (9): 662–5. PMID 8373548.
  21. ↑ Khattak MB, Ismail M, Marwat ZI, Khan F (2012). "Frequency and characterisation of pancytopenia in megaloblastic anaemia". J Ayub Med Coll Abbottabad. 24 (3–4): 53–5. PMID 24669609.
  22. ↑ Germing U, Kobbe G, Haas R, Gattermann N (2013). "Myelodysplastic syndromes: diagnosis, prognosis, and treatment". Dtsch Arztebl Int. 110 (46): 783–90. doi:10.3238/arztebl.2013.0783. PMC 3855821. PMID 24300826.
  23. ↑ Zhang ZN, Liu EK (1991). "[Clinical features of paroxysmal nocturnal hemoglobinuria (PNH) in China as compared with those in United Kingdom]". Zhonghua Nei Ke Za Zhi. 30 (5): 276–9, 317. PMID 1879240.
  24. ↑ Rajput R, Sehgal A, Jain D, Sen R, Gupta A (2012). "Acute parvovirus b19 infection leading to severe aplastic anemia in a previously healthy adult female". Indian J Hematol Blood Transfus. 28 (2): 123–6. doi:10.1007/s12288-011-0112-0. PMC 3332267. PMID 23730023.
  25. ↑ Franck JL, Bouteiller G, Gayrard M, Arlet J (1979). "[D-penicillamine in rheumatoid arthritis : hematological incidents and accidents (author's transl)]". Sem Hop. 55 (27–30): 1325–7. PMID 228408.
  26. ↑ Song IC, Lee HJ, Kim HJ, Bae SB, Lee KT, Yang YJ; et al. (2013). "A multicenter retrospective analysis of the clinical features of pernicious anemia in a Korean population". J Korean Med Sci. 28 (2): 200–4. doi:10.3346/jkms.2013.28.2.200. PMC 3565130. PMID 23400269.
  27. ↑ Birkeland AC, Auerbach AD, Sanborn E, Parashar B, Kuhel WI, Chandrasekharappa SC; et al. (2011). "Postoperative clinical radiosensitivity in patients with fanconi anemia and head and neck squamous cell carcinoma". Arch Otolaryngol Head Neck Surg. 137 (9): 930–4. doi:10.1001/archoto.2011.154. PMC 3343719. PMID 21930984.
  28. ↑ Bauer H (2001). "[Fatal outcome of a multisystemic sarcoidosis in a 54-year-old patient]". Pneumologie. 55 (7): 343–6. doi:10.1055/s-2001-15617. PMID 11481582.
  29. ↑ Palmer K, Green TD, Roberts JL, Sajaroff E, Cooney M, Parrott R; et al. (2007). "Unusual clinical and immunologic manifestations of transplacentally acquired maternal T cells in severe combined immunodeficiency". J Allergy Clin Immunol. 120 (2): 423–8. doi:10.1016/j.jaci.2007.02.047. PMID 17481714.
  30. ↑ Sarkar RN, Banerjee S, Dey S, Saha A, Bhattacharjee P, Banerjee TK; et al. (2009). "Haematological presentation of systemic lupus erythematosus". J Assoc Physicians India. 57: 767–8. PMID 20329445.
  31. ↑ Song S (2011). "A case report: Concurrent chronic myelomonocytic leukemia and T-cell large granular lymphocytic leukemia-type clonal proliferation as detected by multiparametric flow cytometry". Cytometry B Clin Cytom. 80 (2): 126–9. doi:10.1002/cyto.b.20565. PMID 21337493.
  32. ↑ Villano JL, Letarte N, Yu JM, Abdur S, Bressler LR (2012). "Hematologic adverse events associated with temozolomide". Cancer Chemother Pharmacol. 69 (1): 107–13. doi:10.1007/s00280-011-1679-8. PMID 21614470.
  33. ↑ Le Hô H, Barbarot N, Desrues B (2010). "[Pancytopenia in disseminated tuberculosis: Think of macrophage activation syndrome]". Rev Mal Respir. 27 (3): 257–60. doi:10.1016/j.rmr.2010.02.005. PMID 20359619.
  34. ↑ Klimaszyk D, Łukasik-Głebocka M (2011). "[Pancytopenia in the course of acute valproic acid poisoning--case report]". Przegl Lek. 68 (8): 539–42. PMID 22010461.
  35. ↑ Eom TH, Lee HS, Jang PS, Kim YH (2013). "Valproate-induced panhypogammaglobulinemia". Neurol Sci. 34 (6): 1003–4. doi:10.1007/s10072-012-1153-3. PMID 22797722.
  36. ↑ Tun NT, Shukla S, Krishnakurup J, Pappachen B, Krishnamurthy M, Salib H (2014). "An unusual cause of pancytopenia: Whipple's disease". J Community Hosp Intern Med Perspect. 4. doi:10.3402/jchimp.v4.23482. PMC 3992356. PMID 24765256.


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