Pancytopenia: Difference between revisions
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+ | == Background == |
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+ | * Combination of anemia (<120-130), neutropenia (<1.8), and platelet (<150) |
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+ | |||
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+ | |||
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+ | *Bleeding, from [[thrombocytopenia]] |
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+ | |||
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+ | |||
+ | *See also [[aplastic anemia]] |
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+ | *'''Congenital causes''' (chronic pancytopenia) |
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+ | **Telomere diseases: [[dyskeratosis congenita]] (early hair graying, [[pulmonary fibrosis]], [[cirrhosis]]) |
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+ | **[[Fanconi anemia]] (short statue, [[Café-au-lait spot|café-au-lait spots]], skeletal abnormalities, urogenital abnormalities) |
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+ | **[[GATA2 deficiency]] (persistent or unusual infections) |
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+ | **[[CTLA4 deficiency]] (intestinal disease, adenopathy, infections, autoimmune disorders) |
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+ | **[[Schwachman-Diamond syndrome]] (skeletal abnormalities, exocrine pancreatic dysfunction) |
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+ | **[[Wiskott-Aldrich syndrome]] (eczema, recurrent infections, low IgM, elevated IgA/E) |
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+ | *'''Medication-induced''' |
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+ | **[[Immunosuppressive therapy|Immunosuppressants]], [[chemotherapy]], [[Radiation therapy|radiation]] |
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+ | **Antiseizure: [[carbamazepine]], [[levetiracetam]], [[phenytoin]], [[phenobarbital]], [[valproate]] |
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+ | **Gout: [[Allopurinol|Allergic bronchopulmonary aspergillosis]], [[colchicine]] |
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+ | **Antibiotics: coadministration of [[TMP-SMX]] and [[methotrexate]], [[albendazole]], [[chloramphenicol]], [[cidofovir]], [[dapsone]], [[foscarnet]], [[ganciclovir]], [[linezolid]], [[quinine]], [[Sulfonamide|sulfonamides]], [[zidovudine]] |
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+ | **NSAIDs: [[aspirin]], [[diclofenac]], [[ibuprofen]], [[indomethacin]], [[phenylbutazone]], [[Salicylate|salicylates]], [[sulindac]] |
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+ | **Anti-thyroid: [[methimazole]], [[propylthiouracil]] |
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+ | **Cardiovascular: [[aspirin]], [[amiodarone]], [[captopril]], [[lisinopril]], [[nifedipine]], [[quinidine]], [[ticlopidine]] |
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+ | **Diuretics: [[acetazolamide]], [[furosemide]], [[Thiazide|thiazides]] |
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+ | **GI: [[cimetidine]], [[nizatidine]] |
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+ | **Psychiatric: [[bupropion]], [[lithium]], [[valproate]] |
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+ | **Exposures: [[benzene]], [[MDMA]], glue, [[Pesticide|pesticides]], [[Radiation toxicity|radiation]], organic solvents |
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⚫ | |||
**Infection |
**Infection |
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− | ***Viral |
+ | ***Viral |
− | ****[[Epstein-Barr virus]] |
+ | ****[[Epstein-Barr virus]]: chronic active EBV and primary infection that results in [[hemophagocytic lymphohistiocytosis]] |
− | ****[[Parvovirus B19]] |
+ | ****[[Parvovirus B19]]: usually transient, but can also cause pure red cell aplasia |
****[[Cytomegalovirus]], if immunocompromised |
****[[Cytomegalovirus]], if immunocompromised |
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+ | ****[[Hepatitis A virus]] |
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****[[Human immunodeficiency virus]] |
****[[Human immunodeficiency virus]] |
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****[[Dengue virus]] |
****[[Dengue virus]] |
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+ | ***Bacterial |
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− | ****[[Viral hepatitis]] |
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+ | ****[[Mycobacterium tuberculosis]], in the context of disseminated disease |
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− | ***Other |
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− | ****[[ |
+ | ****[[Leptospira]] |
+ | ****[[Brucella]] |
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+ | ****[[Bartonella henselae]] and [[Bartonella quintana]], causing [[bacillary angiomatosis]] involving liver and spleen |
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+ | ****[[Mycoplasma pneumoniae]], associated with fulminant infection and the presence of cold agglutinin disease |
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+ | ****[[Ehrlichia chaffeensis]], causing [[hemophagocytic lymphohistiocytosis]] |
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+ | ***Fungal |
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+ | ****[[Pneumocystis jirovecii]], from bone marrow necrosis |
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+ | ***Parasitic |
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+ | ****[[Visceral leishmaniasis]] |
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**Malignancy |
**Malignancy |
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***[[Hematologic malignancy]] |
***[[Hematologic malignancy]] |
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− | ***[[Solid |
+ | ***[[Solid organ malignancy]] |
****[[Metastases]] |
****[[Metastases]] |
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**Other |
**Other |
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***[[Sarcoidosis]] |
***[[Sarcoidosis]] |
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***[[Amyloidosis]] |
***[[Amyloidosis]] |
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+ | **Nutritional |
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− | **Systemic |
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***[[Vitamin B12 deficiency]] |
***[[Vitamin B12 deficiency]] |
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+ | ***May be from underlying severe caloric restriction, [[eating disorder]], bariatric surgery, [[malabsorption]], or alcohol use |
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− | *Increased destruction |
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+ | *'''Increased destruction with decreased production''': autoimmune disorders, [[PNH]], [[Hematologic malignancy|hematologic malignancies]], [[HLH]], [[sepsis]] |
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− | *Sequestration |
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+ | *'''Increased destruction''' ([[hemolysis]] with increased reticulocytes): [[Evan syndrome]], [[thrombotic microangiopathy]], [[DIC]] |
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+ | *'''Sequestration''' (splenomegaly): [[portal hypertension]], [[cirrhosis]], infections, autoimmune disorders, [[myeloproliferative neoplasm]], [[leukemia]], [[lymphoma]], storage diseases |
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+ | == Investigations == |
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⚫ | |||
+ | * Initial investigations: |
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⚫ | |||
+ | ** Review prior CBCs to determine chronicity |
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− | *Bleeding |
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+ | ** CBC with differential and reticulocyte count |
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⚫ | |||
+ | **Peripheral blood film |
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+ | ***[[Leukoerythroblastic reaction]]: BM infiltration and sepsis |
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+ | ***Blasts: [[AML]] or [[ALL]] |
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+ | ***[[Pelger-Huet anomaly]] or dysplasia: [[MDS]] |
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+ | ***Megaloblastic: [[Vitamin B12 deficiency|B12]] or [[Folate deficiency|folate]] deficiency |
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+ | ***Atypical lymphocytes: viral (such as [[EBV]]) |
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+ | ***Schistocytes: [[DIC]], [[sepsis]], [[TMA]] |
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+ | ** Lytes/creatinine, uric acid, LDH |
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+ | ** Liver panel |
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+ | ** Coags |
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+ | ** Iron studies |
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+ | ** ESR/[[CRP]] |
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+ | * Consider the following, based on initial workup: |
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+ | ** Copper, zinc, B12/MMA, folate |
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+ | ** [[HLH]] workup |
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+ | ** Infectious workup |
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+ | ** Haptoglobin |
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+ | ** [[HIV]], [[EBV]], [[CMV]], [[HBV]], [[HCV]], [[HHV6]], [[parvovirus]] |
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+ | ** ANA, ENA |
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+ | ** DIC panel |
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+ | * Then consider the following, based on initial workup: |
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+ | ** Flow cytometry |
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+ | ** Tuberculosis or fungal workup |
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+ | ** US/CT/MRI/PET-CT |
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+ | ** Bone marrow aspirate and biopsy |
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[[Category:Hematology]] |
[[Category:Hematology]] |
Latest revision as of 12:14, 28 August 2023
Background
- Combination of anemia (<120-130), neutropenia (<1.8), and platelet (<150)
Clinical Manifestations
- Increased infections, from leukopenia
- Bleeding, from thrombocytopenia
- Angina and other hypoxemic symptoms, from anemia
Differential Diagnosis
- See also aplastic anemia
- Congenital causes (chronic pancytopenia)
- Telomere diseases: dyskeratosis congenita (early hair graying, pulmonary fibrosis, cirrhosis)
- Fanconi anemia (short statue, café-au-lait spots, skeletal abnormalities, urogenital abnormalities)
- GATA2 deficiency (persistent or unusual infections)
- CTLA4 deficiency (intestinal disease, adenopathy, infections, autoimmune disorders)
- Schwachman-Diamond syndrome (skeletal abnormalities, exocrine pancreatic dysfunction)
- Wiskott-Aldrich syndrome (eczema, recurrent infections, low IgM, elevated IgA/E)
- Medication-induced
- Immunosuppressants, chemotherapy, radiation
- Antiseizure: carbamazepine, levetiracetam, phenytoin, phenobarbital, valproate
- Gout: Allergic bronchopulmonary aspergillosis, colchicine
- Antibiotics: coadministration of TMP-SMX and methotrexate, albendazole, chloramphenicol, cidofovir, dapsone, foscarnet, ganciclovir, linezolid, quinine, sulfonamides, zidovudine
- NSAIDs: aspirin, diclofenac, ibuprofen, indomethacin, phenylbutazone, salicylates, sulindac
- Anti-thyroid: methimazole, propylthiouracil
- Cardiovascular: aspirin, amiodarone, captopril, lisinopril, nifedipine, quinidine, ticlopidine
- Diuretics: acetazolamide, furosemide, thiazides
- GI: cimetidine, nizatidine
- Psychiatric: bupropion, lithium, valproate
- Exposures: benzene, MDMA, glue, pesticides, radiation, organic solvents
- Decreased production
- Infection
- Viral
- Epstein-Barr virus: chronic active EBV and primary infection that results in hemophagocytic lymphohistiocytosis
- Parvovirus B19: usually transient, but can also cause pure red cell aplasia
- Cytomegalovirus, if immunocompromised
- Hepatitis A virus
- Human immunodeficiency virus
- Dengue virus
- Bacterial
- Mycobacterium tuberculosis, in the context of disseminated disease
- Leptospira
- Brucella
- Bartonella henselae and Bartonella quintana, causing bacillary angiomatosis involving liver and spleen
- Mycoplasma pneumoniae, associated with fulminant infection and the presence of cold agglutinin disease
- Ehrlichia chaffeensis, causing hemophagocytic lymphohistiocytosis
- Fungal
- Pneumocystis jirovecii, from bone marrow necrosis
- Parasitic
- Viral
- Malignancy
- Other
- Nutritional
- Vitamin B12 deficiency
- May be from underlying severe caloric restriction, eating disorder, bariatric surgery, malabsorption, or alcohol use
- Infection
- Increased destruction with decreased production: autoimmune disorders, PNH, hematologic malignancies, HLH, sepsis
- Increased destruction (hemolysis with increased reticulocytes): Evan syndrome, thrombotic microangiopathy, DIC
- Sequestration (splenomegaly): portal hypertension, cirrhosis, infections, autoimmune disorders, myeloproliferative neoplasm, leukemia, lymphoma, storage diseases
Investigations
- Initial investigations:
- Review prior CBCs to determine chronicity
- CBC with differential and reticulocyte count
- Peripheral blood film
- Lytes/creatinine, uric acid, LDH
- Liver panel
- Coags
- Iron studies
- ESR/CRP
- Consider the following, based on initial workup:
- Then consider the following, based on initial workup:
- Flow cytometry
- Tuberculosis or fungal workup
- US/CT/MRI/PET-CT
- Bone marrow aspirate and biopsy