Pancytopenia: Difference between revisions
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==Differentiating [Disease] from Other Diseases== | ==Differentiating [Disease] from Other Diseases== | ||
Pancytopenia must be differentiated from a pseudo-syndromes like pseudo-thrombocytopenia, which is a laboratory artifact from platelet clumping. | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
Pancytopenia affects males and females equally. However, the underlying etiologies of pancytopenia can have a gender predilection. | |||
==Risk Factors== | ==Risk Factors== | ||
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==Diagnosis== | ==Diagnosis== | ||
===Diagnostic Criteria=== | ===Diagnostic Criteria=== | ||
The diagnosis of pancytopenia is made when all of the following criteria are fulfilled: | |||
*Anemia as defined by hemoglobin level < 12 grams per [[deciliter]] (g/dl) | |||
*Leukopenia as defined by leukocyte count < 4000 per [[microliter]] | |||
*Thrombocytopenia as defined by platelet count < 150000 per [[microliter]] | |||
===History and Symptoms=== | ===History and Symptoms=== | ||
Symptoms are pancytopenia are related to decrease in erythrocytes, leukocytes, and platelets. | |||
Decrease in erythrocytes causes fatigue, shortness of breath, decreased exercise tolerance, and pallor. | |||
Decrease in leukocytes causes infection, which can affect a multitude of organ systems including the central nervous system, lungs, abdomen, urinary tract, kidneys, and skin. | |||
Decrease in platelets causes mucocutaneous bleeding, typically of the nose, mouth, gastrointestinal tract, or genitourinary tract. | |||
===Physical Examination=== | ===Physical Examination=== | ||
Revision as of 05:12, 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
- Aplastic anemia [1]
- Folate deficiency
- Leishmaniasis
- Leukemia: This can be myeloid or lymphoid, and each of these can be acute or chronic.
- Megaloblastic anemia [1]
- Myelodysplastic syndrome
- Paroxysmal nocturnal hemoglobinuria
- Viral infections e.g. (HIV most common)
- Vitamin B12 deficiency
- Copper deficiency
- Zinc deficiency
Causes by Organ System
Causes in Alphabetical Order
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
Causes by Mode of Inheritance
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
Differentiating [Disease] from Other Diseases
Pancytopenia must be differentiated from a pseudo-syndromes like pseudo-thrombocytopenia, which is a laboratory artifact from platelet clumping.
Epidemiology and Demographics
Pancytopenia affects males and females equally. However, the underlying etiologies of pancytopenia can have a gender predilection.
Risk Factors
The risk factors of pancytopenia are related to the underlying cause. For example, leukemia-mediated pancytopenia can be related to risk factors such as chemical exposure, radiation, or family history.
Screening
There are no suggested screening tests for pancytopenia. The United States Preventive Services Task Force (USPSTF) does not have any recommendations for screening for pancytopenia.
Natural History, Complications, and Prognosis
Natural History
The natural history of pancytopenia is dictated by the pathophysiology of the under etiology. For example, viral-mediated pancytopenia is typically short-lived, pending clearance of the virus. Drug-induced pancytopenia typically resolves after discontinuing of the culprit drug and the drug has been metabolized by the body. Leukemia-mediated pancytopenia is usually a more long-term process, as marrow replacement by leukemia cells is difficult to overcome unless the leukemia is treated and the patient is in remission.
Complications
Complications of pancytopenia relate to deficits of the cell types that are affected. Decrease in erythrocytes causes fatigue and pallor due to decrease in oxygen delivery to tissue beds. Decrease in leukocytes and leukocyte subsets causes infections, which can be viral, bacteria, fungal, or parasitic. Decrease in thrombocytes causes bleeding, which is typically mucosal, given loss of the ability of platelets to create a hemostatic plug.
Prognosis
The prognosis of pancytopenia is related to the underlying etiology. For example, patients with unfavorable-risk leukemia will likely have a poor prognosis from a pancytopenia perspective. Patients with viral-mediated pancytopenia have a prognosis that is determined by the natural history of the virus. Epstein-Barr virus (EBV)-related pancytopenia can have a good prognosis if EBV resolves. Drug-induced pancytopenia has a favorable prognosis, as discontinuation of the offending agent can typically reverse the pancytopenia.
Diagnosis
Diagnostic Criteria
The diagnosis of pancytopenia is made when all of the following criteria are fulfilled:
- Anemia as defined by hemoglobin level < 12 grams per deciliter (g/dl)
- Leukopenia as defined by leukocyte count < 4000 per microliter
- Thrombocytopenia as defined by platelet count < 150000 per microliter
History and Symptoms
Symptoms are pancytopenia are related to decrease in erythrocytes, leukocytes, and platelets. Decrease in erythrocytes causes fatigue, shortness of breath, decreased exercise tolerance, and pallor. Decrease in leukocytes causes infection, which can affect a multitude of organ systems including the central nervous system, lungs, abdomen, urinary tract, kidneys, and skin. Decrease in platelets causes mucocutaneous bleeding, typically of the nose, mouth, gastrointestinal tract, or genitourinary tract.
Physical Examination
Key components of the physical exam include assessment of the conjunctiva, oral and nasal mucosa, lymph nodes (cervical, axillary, supraclavicular, inguinal), spleen size, liver size, and skin.
The anemia component of pancytopenia can cause conjunctival pallor, mucosal pallor, and skin pallor. The leukopenia component of pancytopenia can cause variable findings depending on whether infection is present. Exam findings can include lymphadenopathy, egophony, coarse breath sounds, malodorous urine, suprapubic tenderness, costovertebral tenderness, abdominal tenderness, skin erythema, and/or skin purulence.
Laboratory Findings
Laboratory findings in pancytopenia are, by definition: Hemoglobin level < 12 grams per deciliter (g/dl) Leukocyte count < 4000 per microliter Platelet count < 150000 per microliter
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
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