Pancytopenia

Template:Pancytopenia Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

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 1) bone marrow suppression caused by either insufficient production (aplastic anemia), inability of cells or mature (myelodysplasia), or replacement of normal bone marrow with fibrosis (myelofibrosis) or 2) peripheral sequestration/destruction that is not related to the bone marrow (e.g. splenomegaly or hypersplenism). HIV (Human Immunodeficiency Virus) is itself a cause for pancytopenia. Chemotherapy is associated with pancytopenia. Pancytopenia usually requires a bone marrow biopsy in order to distinguish among different causes.

Causes

Common Causes

• Aplastic anemia

• Myelodysplastic syndrome

• Leukemia

• Leishmaniasis

• Severe Folate or Vitamin B12 deficiency

• Paroxysmal nocturnal hemoglobinuria (Urine test)

• Overwhelming viral infections (HIV most common)

Causes by Pathophysiology

Bone Marrow Failure

• Insufficient production (aplastic anemia)
• Inability of cells or mature (myelodysplasia)
• Replacement of normal bone marrow with fibrosis (myelofibrosis)

Peripheral Sequestration/Destruction

• Splenomegaly
• Hypersplenism

Congenital

• Cartilage hair hypoplasia
• Diamond-Blackfan syndrome
• Dubowitz syndrome
• Dyskeratosis congenita
• Familial aplastic anemia
• Fanconi's anemia
• Pearson syndrome
• Schwachman-Diamond syndrome
• TAR syndrome

Acquired

• Albers-Schonberg disease
• Banti's Syndrome
• Bone marrow tumor
• Cirrhosis
• Drugs/Toxins
• Felty's Syndrome
• Gaucher's Disease
• Graft-versus-host disease
• Infections
• Kala-Azar
• Leukemia
• Lymphoma
• Lymphoproliferative Disorders
• Myelodysplastic syndrome
• Myelofibrosis
• Niemann-Pick Disease
• Osteoporosis
• Pernicious anemia
• Reticulosis
• Sarcoidosis
• Thymoma
• Tuberculosis

Causes by Organ System

Cardiovascular	No underlying causes
Chemical/Poisoning	No underlying causes
Dental	No underlying causes
Dermatologic	No underlying causes
Drug Side Effect	Acetaminophen and Oxycodone, Aclarubicin, Albendazole, Alemtuzumab, Alkylating antineoplastic agent, Alkylators, Arsenicals, Auranofin, Azathioprine, Aztreonam, Boceprevir, Busulphan, carbamazepine, Carboplatin, Cefadroxil, Ceftazidime, Certolizumab pegol, Chlorpromazine, Chloramphenicol, Chlorpropamide, Cidofovir, Clomipramine, Colchicine toxicity, Cytotoxic drugs and radiation, Dactinomycin, Doxorubicin, Ethosuximide, Febuxostat, Flucytosine, Gold, Gemifloxacin mesylate, Idarubicin, indomethacin , 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, Tolbutamide, Trifluoperazine, Thiothixene, Valganciclovir hydrochloride
Ear Nose Throat	No underlying causes
Endocrine	Thyroid carcinoma
Environmental	Tobacco use, Environmental/occupational (eg, benzene)
Gastroenterologic	Portal hypertension, Intestinal lymphangiectasia, Hepatitis
Genetic	Ataxia telangiectasia, Barth syndrome, Chediak-Higashi disease, defect in synthesis of adenosylcobalamin, DNA repair deficiencies, Down syndrome, Dubowitz syndrome, familial monosomy 7, Fanconi anaemia, genotoxic therapy, glutathione synthase deficiency, glycogen storage diseases, mutagen exposure, mutagen-detoxification (GSTq1-null), neurofibromatosis 1, Niemann-Pick disease, Nijmege breakage syndrome, Noonan syndrome, organic acid metabolism defect, Pearson syndrome, Seckel syndrome, Severe combined immunodeficiency, T- B+ due to JAK3 deficiency, trisomy 8 mosaicism, Wiskott-Aldrich syndrome
Hematologic	Sickle cell disease, Reticular dysgenesis, Paroxysmal nocturnal haemoglobinuria, Non-Hodgkin lymphoma, Myelpathic anemia, Myeloma, Myelofibrosis, Myelodysplastic syndrome, Megaloblastic anaemia, Malignant histiocytosis, Leucoerythroblastic anaemia, Large granular lymphocytic leukemia, Langerhans cell histiocytosis, Ineffective erythropoiesis, Hypoplastic myelodysplastic syndromes , Hypersplenism, Hodgkin lymphoma, Histiocytosis X, Hepatosplenic T-cell lymphoma, Hemoglobin H disease, Hematopoietic cell transplantation, Hairy cell leukaemia, Haemoglobin SC disease, Haemoglobin E disease, Graft versus host disease, Familial myelofibrosis, Familial histiocytic reticulosis, Eosinophilic fasciitis , Cyclical neutropenia, Congenital neutropenia (Kostmann's or Shwachman-Diamond syndrome), Common variable hypogammaglobulinaemia, Chronic myeloid leukaemia, Chronic lymphocytic leukaemia, Bleeding (Excessive), Autoimmune lymphoproliferative syndrome type 2, Autoimmune lymphoproliferative syndrome type 1, Aplastic anemia, Amegakaryocytic thrombocytopenia, Aggressive NK-cell leukemia, Acute lymphoblastic leukemia
Iatrogenic	No underlying causes
Infectious Disease	Q fever, Parvovirus B19 infection, Oklahoma tick fever, Mycobacterium tuberculosis, Legionella pneumophila, Lassa fever, Human immunodeficiency virus , Epstein-Barr virus Seronegative, Dengue, Brucellosis
Musculoskeletal/Orthopedic	No underlying causes
Neurologic	No underlying causes
Nutritional/Metabolic	Vitamin C deficiency, Vitamin B12 deficiency, Marasmus, Malabsorption syndrome, Kwashiorkor, Iron deficiency, Intrinsic factor deficiency, Folate deficiency, Copper deficiency
Obstetric/Gynecologic	Pregnancy
Oncologic	Thymoma , Prostate cancer, Metastatic neoplasm, Melanoma, Germ cell tumors (embryonal dysgenesis), Ewing sarcoma, Chondrosarcoma, Cancer treatment
Ophthalmologic	No underlying causes
Overdose/Toxicity	No underlying causes
Psychiatric	Anorexia nervosa
Pulmonary	No underlying causes
Renal/Electrolyte	Renal failure, chronic
Rheumatology/Immunology/Allergy	Systemic lupus erythematosus, Rheumatoid disease, Osteopetrosis autosomal recessive 2, Osteopetrosis lethal Osteoclastoma, Ankylosing spondylitis
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	Topoisomerase II interactive agents, Plummer-Vinson syndrome, Obesity, Idiopathic , Glue vapors, Dyskeratosis congenita Shwachman-Diamond syndrome, Diamond-Blackfan anemia, Castleman disease, Bloom's syndrome

Causes in Alphabetical Order

• Aclarubicin

• Acute lymphoblastic leukemia

• Aggressive NK-cell leukemia

• Albendazole

• Alemtuzumab

• Alkylating antineoplastic agent

• Alkylators

• Amegakaryocytic thrombocytopenia

• Ankylosing spondylitis

• Anorexia nervosa

• Aplastic anemia

• Arsenicals

• Ataxia telangiectasia

• Auranofin

• Autoimmune lymphoproliferative syndrome type 1

• Autoimmune lymphoproliferative syndrome type 2

• Azathioprine

• Aztreonam

• Benzene Solvents

• Beta-emitters (eg, radioactive P-32)

• Bleeding (Excessive)

• Bloom's syndrome

• Boceprevir

• Brucellosis

• Busulphan

• Cancer treatment

• carbamazepine

• Carboplatin

• Castleman disease

• Cefadroxil

• Ceftazidime

• Chediak-Higashi disease

• Chloramphenicol

• Chlorpropamide

• Chondrosarcoma

• Chronic lymphocytic leukaemia

• Chronic myeloid leukaemia

• Cidofovir

• Clomipramine

• Colchicine toxicity

• Common variable hypogammaglobulinaemia

• Congenital neutropenia (Kostmann's or Shwachman-Diamond syndrome)

• Copper deficiency

• Cyclical neutropenia

• Cytotoxic drugs and radiation

• Defect in synthesis of adenosylcobalamin

• Dengue

• Diamond-Blackfan anemia

• DNA repair deficiencies

• Down syndrome (trisomy 21)

• Doxorubicin

• Dyskeratosis congenita Shwachman-Diamond syndrome

• Environmental/occupational (eg, benzene)

• Eosinophilic fasciitis

• Epstein-Barr virus Seronegative

• Ewing sarcoma

• Familial histiocytic reticulosis

• Familial monosomy 7

• Familial myelofibrosis

• Fanconi anaemia

• Flucytosine

• Folate deficiency

• Genotoxic therapy

• Germ cell tumors (embryonal dysgenesis)

• Glue vapors

• Glutathione synthase deficiency

• Gold

• Graft versus host disease

• Haemoglobin E disease

• Haemoglobin SC disease

• Hairy cell leukaemia

• Hematopoietic cell transplantation

• Hemoglobin H disease

• Hepatitis

• Hepatosplenic T-cell lymphoma

• Histiocytosis X

• Hodgkin lymphoma

• Human immunodeficiency virus

• Hypersplenism

• Hypoplastic myelodysplastic syndromes

• Idarubicin

• Idiopathic

• indomethacin

• Ineffective erythropoiesis

• Intestinal lymphangiectasia

• Infliximab

• Intrinsic factor deficiency

• Iron deficiency

• Kwashiorkor

• Langerhans cell histiocytosis

• Large granular lymphocytic leukemia

• Lassa fever

• Legionella pneumophila

• Leucoerythroblastic anaemia

• Malabsorption syndrome

• Malignant histiocytosis

• Marasmus

• Megaloblastic anaemia

• Melanoma

• Metastatic neoplasm

• Mutagen exposure

• Mutagen-detoxification (GSTq1-null)

• Mycobacterium tuberculosis

• Myelodysplastic syndrome

• Myelofibrosis

• Myeloma

• Myelpathic anemia

• Neurofibromatosis 1

• Niemann-Pick disease

• Non steroidal anti-inflammatory drugs

• Non-Hodgkin lymphoma

• Obesity

• Oklahoma tick fever

• Osteoclastoma

• Osteopetrosis lethal

• Osteopetrosis, autosomal recessive 2

• Osteosarcoma

• Oxaprozin

• Paroxysmal nocturnal haemoglobinuria

• Parvovirus B19 infection

• phenacemide

• phenylbutazone

• Plummer-Vinson syndrome

• Portal hypertension

• Pregnancy

• Prostate cancer

• Propylthiouracil

• Pyrimethamine

• Q fever

• Renal failure, chronic

• Reticular dysgenesis

• Rheumatoid disease

• Severe combined immunodeficiency, T- B+ due to JAK3 deficiency

• Sickle cell disease

• Sulfonamides

• Systemic lupus erythematosus

• Tamoxifen

• Thymoma

• Thyroid carcinoma

• Tobacco use

• Topoisomerase II interactive agents

• Trisomy 8 mosaicism

• Valganciclovir hydrochloride

• Vitamin B12 deficiency

• Vitamin C deficiency

• Wiskott-Aldrich syndrome

• Xeroderma pigmentosum