Presentation
AB - Clericuzio-type poikiloderma with neutropenia is a well-defined nosological entity, but despite a remarkable number of clinical reports, no long term follow-up data has been presented to date regarding patients with this rare condition. [moh-it.pure.elsevier.com]
At age 19 years she presented with edema of the limbs, proteinuria and renal failure. Renal amyloidosis AA was diagnosed by biopsy. [pubmed.ncbi.nlm.nih.gov]
When to Order a CBC A CBC is not ordered routinely for well children examined in the pediatrician's office or when children present with common febrile illnesses such as upper respiratory tract infections or otitis media. [pedsinreview.aappublications.org]
Clinical History Patients with neutropenia often present with infection. [odlarmed.com]
Presents with severe infections in the first month of life. [learn.pediatrics.ubc.ca]
Immune System
- Splenomegaly
Agranulocytic angina Infantile genetic agranulocytosis Kostmann disease Neutropenia: NOS congenital cyclic drug-induced periodic splenic (primary) toxic Neutropenic splenomegaly Werner-Schultz disease Coding-Hint Use additional external cause code [icd.who.int]
[…] organs and certain disorders involving the immune mechanism D70 ICD-10-CM Diagnosis Code D70 Neutropenia 2016 2017 2018 2019 Non-Billable/Non-Specific Code Includes agranulocytosis decreased absolute neurophile count (ANC) Type 1 Excludes neutropenic splenomegaly [icd10data.com]
Splenomegaly and hepatomegaly38 Splenomegaly children20 Steatohepatitis Stridor pediatric age4 Stroke36 Stroke pediatric age23 Stroke young adult causes1 St segment elevations nonischemic Sudden death pediatric age4 Sudden death young athlete Swollen [books.google.com]
Splenomegaly : Enlargement of the spleen can cause a reduction if the white blood cell count. Cyclic Neutropenia : This is a rare inherited condition (autosomal dominant) where patient have several days of a low white blood cell count every 3 weeks. [sites.google.com]
Splenic sequestration: The degree of neutropenia resulting from this process is proportional to the severity of the splenomegaly and the bone marrow’s ability to compensate for the reduction in circulating bands and neutrophils. [odlarmed.com]
Hematological
- Hemophilia A
Such diseases might include hemophilia, blood clots, other bleeding disorders and blood cancers such as leukemia, multiple myeloma, and lymphoma. [en.wikipedia.org]
[…] deficiency / Activated protein C resistance / Protein S deficiency / Factor V Leiden - Hyperprothrombinemia acquired: DIC ( Congenital afibrinogenemia, Purpura fulminans ) - autoimmune ( Antiphospholipid ) Other Essential thrombocytosis - clotting factor: Hemophilia [wikidoc.org]
Entire Body System
- Anemia
Nutritional Iron deficiency anemia ( Plummer-Vinson syndrome ), Megaloblastic anemia ( Pernicious anemia ) Hemolytic Hereditary enzyme: G6PD Deficiency - Pyruvate kinase deficiency - Triosephosphate isomerase deficiency hemoglobin: Thalassemia - Sickle-cell [wikidoc.org]
WT syndrome, ataxia-pancytopenia syndrome, familial platelet disorder with predisposition to acute myelogenous leukemia Predominantly anemia Diamond-Blackfan anemia, congenital dyserythropoietic anemias, sideroblastic anemia Predominantly neutropenia [sickkids.ca]
Bone marrow failure and Myelodysplasia disorders include: Aplastic anemia Fanconi anemia Shwachman-Diamond syndrome Congenital amegakaryocytic thrombocytopenia Dyskeratosis congenital Congenital hair hypoplasia Pearson disease Diamond Blackfan anemia [yellowballoon.org]
Thiamine-responsive anemia in DIDMOAD syndrome (see pages 64-65) g. Barth syndromec (see page 223) 11. Bone marrow aplasia a. Fanconi anemia b. Familial congenital aplastic anemia without anomalies c. [arthritisresearch.us]
- Asymptomatic
At conception, each sib of an affected individual has a 25% chance of being affected, a 50% chance of being an asymptomatic carrier, and a 25% chance of being unaffected and not a carrier. [ncbi.nlm.nih.gov]
If the patient is asymptomatic and does not have a history of infections, they do not require therapy in response to their neutrophil number. [cancertherapyadvisor.com]
The results from the trials reported in the current manuscript do confirm that neutropenia occurring after vaccination is normally transient, clinically asymptomatic and do not reveal any association with age and gender of vaccinees. [journals.plos.org]
Patients are typically asymptomatic, and the infections are mild. No specific therapy is required. Nonfamilial chronic benign neutropenias: Mild infections with a benign course typify this disorder. [odlarmed.com]
Patients are typically asymptomatic, and the infections are mild. No specific therapy is required. In nonfamilial chronic benign neutropenias, mild infections with a benign course typify this disorder. [emedicine.medscape.com]
- Splenectomy
Monocytopenia Glucocorticoid administration Infections associated with endotoxemia Acute hemorrhage Malignant neoplasms Carcinoma Sarcoma Lymphoma Connective tissue diseases Rheumatic fever Rheumatoid arthritis Inflammatory bowel disease Hematologic diseases Splenectomy [arthritisresearch.us]
Autologous 111 In-labelled platelet sequestration studies in patients with primary immune thrombocytopenia (ITP) prior to splenectomy: a report from the United Kingdom ITP Registry. Br J Haematol 2010;151:477–487. 12. [mjhid.org]
Splenectomy shows an initial response, but neutropenia may recur in 10-20% of patients. Treatment is directed toward rheumatoid arthritis. [odlarmed.com]
Effective splenectomy in agranulocytosis associated with systemic lupus erythematosus. J Rheumatol. 1997 Jan. 24(1):234-5. [Medline]. Palmblad J, Nilsson CC, Höglund P, Papadaki HA. How we diagnose and treat neutropenia in adults. [emedicine.medscape.com]
- Hodgkin Lymphoma
Hodgkin's Lymphoma and non Hodgkin's lymphoma : Patients with these conditions can present with a low white blood cell count HIV and AIDS : Because the HIV virus affects lymphocytes which is a type of white blood cell, HIV positive patients can have low [sites.google.com]
Myelodysplastic Syndromes Myeloproliferative Neoplasms Neuroendocrine and Adrenal Tumors Non-Hodgkin's Lymphomas Primary Cutaneous Lymphomas Non-Melanoma Skin Cancers Dermatofibrosarcoma Protuberans Squamous Cell Skin Cancer Non-Small Cell Lung Cancer [nccn.org]
Full Title: A randomized, parallel group, multi-centre phase-2 study of GX-G3 compared with pegfilgrastim as an adjunct to chemotherapy in patients with Non-Hodgkin’s Lymphoma Medical condition: Neutropenia Disease: Version SOC Term Classification Code [clinicaltrialsregister.eu]
Routine Primary Prophylaxis for Febrile Neutropenia with Biosimilar Granulocyte Colony-Stimulating Factor (Nivestim) or Pegfilgrastim Is Cost Effective in Non-Hodgkin Lymphoma Patients undergoing Curative-Intent R-CHOP Chemotherapy. [emedicine.medscape.com]
Respiratoric
- Stridor
Small intestine ulceration Smell disturbance Spastic paraplegias Spinal cord compression epidural Spinal cord dysfunction nontraumatic2 Spinal cord ischemic syndromes Splenomegaly Splenomegaly and hepatomegaly38 Splenomegaly children20 Steatohepatitis Stridor [books.google.com]
Gastrointestinal
- Failure to Thrive
Fred Ferri’s popular "5 books in 1" format provides quick guidance on menorrhagia, Failure to Thrive (FTT), Cogan’s syndrome, and much more. [books.google.com]
Diagnosis is usually made during the first months of life as SDS babies exhibit a failure to thrive, feeding problems and lowered blood cell counts. [6] Chediak-Higashi Syndrome Chediak-Higashi Syndrome is an inherited disorder that is a systemwide disease [tau.ac.il]
"Probably same condition as idiopathic autoimmune neutropenia. cRare genetic disorder consisting of neutropenia, cardiomyopathy, muscle weakness, failure to thrive and growth retardation. isolated leukopenia resulting from a decrease in all classes of [arthritisresearch.us]
Other features include anemia, thrombocytopenia, developmental and mental retardation, diarrhea, failure to thrive, and recurrent otitis media and pneumonia. The chromosomal mutation is located at locus 7qll. [clinicaladvisor.com]
Liver, Gall & Pancreas
- Hepatomegaly
[…] inner ear deafness, venous angiectasias of trunk and limbs SCN5 (VPS45 deficiency) VPS45 AR Bone marrow fibrosis, nephromegaly, extramedullary hematopoiesis Glycogen storage disease type 1b G6PT1 AR Fasting hypoglycemia, lactic acidosis, hyperlipidemia, hepatomegaly [arupconsult.com]
Hepatomegaly by shape of liver38 Hermaphroditism4 Hiccups21 Hip pain without obvious fracture2 Hip pain children26 Hip pain in different age groups8 Hirsutism HIV infection anorectal lesions26 HIV infection chest radiographic abnormalities26 HIV infection [books.google.com]
Cardiovascular
- Tachycardia
If signs or symptoms of a severe illness are apparent (fever, hypotension, tachycardia, ANC Is the neutropenia chronic? A review of previous CBCs will identify whether this condition is new or chronic. [mdedge.com]
[…] diagnostic approach Tachycardia narrow complex Tachycardia wide complex Testicular mass Thoracic outlet syndrome ICD9CM ICD9CM Diagnostic imaging ICD9CM Diagnostic imaging Thrombotic thrombocytopenic purpura and hemolyticuremic syndrome Thyroid nodule [books.google.com]
These patients do not generate increased leukocyte counts with infection, but they have fevers and other symptoms, such as tachycardia, when infected. A history of autoimmune diseases may be associated with antineutrophil antibodies. [emedicine.medscape.com]
- Cyanosis
[…] disease Constipation ICD9CM Contraceptive use oral Convulsive disorder suspected pediatric age Corneal disorders ICD9CM Coronary artery disease unstable Cough chronic Creatine kinase elevation Cushings syndrome Cutaneous microvascular occlusion syndromes Cyanosis [books.google.com]
Skin
- Petechiae
Other sequelae may reflect concurrent pancytopenia (which may increase the patient’s risk for spontaneous bleeding), with anemic symptoms (eg, fatigue, weakness, dyspnea on exertion) and symptoms of thrombocytopenia (eg, petechiae, purpura, epistaxis) [odlarmed.com]
Other sequelae may reflect concurrent pancytopenia, such as anemic symptoms (eg, fatigue, weakness, dyspnea on exertion) and manifestations of thrombocytopenia (eg, petechiae, purpura, epistaxis). [emedicine.medscape.com]
Musculoskeletal
- Muscle Weakness
"Probably same condition as idiopathic autoimmune neutropenia. cRare genetic disorder consisting of neutropenia, cardiomyopathy, muscle weakness, failure to thrive and growth retardation. isolated leukopenia resulting from a decrease in all classes of [arthritisresearch.us]
[…] disease34a Muscle weakness Muscle weakness lower motor neuron versus upper motor neuron40 Mydriasis Myelin disorders Myelopathy and myelitis36 Myocardial ischemia36 Myoclonus Myopathies infectious Myopathies toxic1 Nail clubbing Nail horizontal white [books.google.com]
- Osteoporosis
Optic atrophy34a Oral mucosa erythematous lesions9 Oral mucosa pigmented lesions9 Oral mucosa punctate erosive lesions9 Oral mucosa white lesions9 Oral ulcers acute Orbital lesions calcified Orbital lesions cystic Orgasm dysfunction12 Orofacial pain Osteoporosis [books.google.com]
Neurologic
- Tremor
Taste and smell loss1 Telangiectasia Tendinopathy26 Testicular failure10 Testicular pain Testicular size variations10 Tetanus26 Thrombocytopenia Thrombocytopenia in pregnancy20 Thrombocytosis Thyromegaly Tickrelated infections Tics Torsades de pointes22 Tremor [books.google.com]
- Paresthesia
[…] druginduced2 Pancytopenia20 Papilledema Papulosquamous diseases14 Paraneoplastic neurologic syndromes Paraneoplastic syndromes endocrine36 Paraneoplastic syndromes nonendocrine36 Paraparesis acute or subacute34a Paraparesis chronic progressive34a Paraplegia Paresthesias [books.google.com]
Workup
Obtaining a history of infections may aid in the current diagnostic workup. Obtaining a careful drug history may reveal the offending agent and spare the patient from an extensive diagnostic workup. [odlarmed.com]
Obtaining a history of infections may aid in the current diagnostic workup. A history of periodically recurring infections is suggestive of cyclic neutropenia. [emedicine.medscape.com]
Segel's Pediatrics In Review article (see references) is a good guide in terms of what interventions and workup may be needed: The decision surrounding treatment and potential hospitalization depends on the l ikelihood of bacterial infection, the location [pedclerk.bsd.uchicago.edu]
The workup of such patients should include a complete screening for differential diagnosis, cytogenetic analysis, an expert review of the bone marrow smears, and the help of emerging diagnostic tools such as flow cytometry and molecular biology. [mjhid.org]
Click figure to enlarge Once acquired neutropenia is ruled out, the workup should establish the type of inherited neutropenia. [psychiatrist.com]
Serum
- Cytopenia
[…] worsens or if a second cytopenia develops. [mjhid.org]
[…] non-syndromic thrombocytopenia Dyserythropoietic anemia with thrombocytopenia Familial thrombocytopenia with propensity for leukemia Myelodysplastic syndromes MFM disorders comprise a large number of genetic and acquired conditions with life threatening cytopenias [yellowballoon.org]
We decided to look at this association amongst elderly individuals, an age group particularly vulnerable to cytopenias [ 19, 31, 32 ]. [oncotarget.com]
- Thrombocytosis
III deficiency - Protein C deficiency / Activated protein C resistance / Protein S deficiency / Factor V Leiden - Hyperprothrombinemia acquired: DIC ( Congenital afibrinogenemia, Purpura fulminans ) - autoimmune ( Antiphospholipid ) Other Essential thrombocytosis [wikidoc.org]
Medical condition: Polycythemia Vera Essential thrombocytosis Disease: Version SOC Term Classification Code Term Level 15.0 10027433 - Metabolism and nutrition disorders 10022958 Iron and trace metal metabolism disorders HLGT 15.0 10005329 - Blood and [clinicaltrialsregister.eu]
Leukocytosis, erythrocytosis and thrombocytosis were defined, per established criteria, as WBC>11x10 3 /ul, hemoglobin >16.5 mg/dl in women and >18.5 mg/dl in men, and platelet count > 450x10 3 /ul, respectively. [oncotarget.com]
Sudden death young athlete Swollen limb Tall stature27 Tardive dyskinesia13 Taste and smell loss1 Telangiectasia Tendinopathy26 Testicular failure10 Testicular pain Testicular size variations10 Tetanus26 Thrombocytopenia Thrombocytopenia in pregnancy20 Thrombocytosis [books.google.com]
- Macrocytic Anemia
Cartilage-hair hypoplasia presents with cell-mediated immunity defects, macrocytic anemia, gastrointestinal disease, and dwarfism. It also shows a predisposition to cancer, especially lymphoma. [odlarmed.com]
CHH presents with cell-mediated immunity defects, macrocytic anemia, gastrointestinal disease, and dwarfism. It also shows a predisposition to cancer, especially lymphoma. The treatment is bone marrow transplantation. [emedicine.medscape.com]
Anemia in newborn Anemia of chronic disease Anemia hemolytic Anemia macrocytic Anemia microcytic Anemia with reticulocytosis Anisocoria Anorexia Antinuclear antibody testing Aortic stenosis diagnostic approach Aortic stenosis management Aplastic anemia [books.google.com]
- Neutrophil Count Decreased
O f t hese, 12 articles described patients with benign ethnic neutropenia whose neutrophil count decreased during treatment with clozapine. [psychiatrist.com]
- Microcytic Anemia
Anemia in newborn Anemia of chronic disease Anemia hemolytic Anemia macrocytic Anemia microcytic Anemia with reticulocytosis Anisocoria Anorexia Antinuclear antibody testing Aortic stenosis diagnostic approach Aortic stenosis management Aplastic anemia [books.google.com]
Treatment
Treatment Basic information Diagnosis Treatment Basic information Diagnosis Treatment Pearls considerations Basic information Diagnosis Treatment Pearls considerations Basic information Diagnosis Treatment Pearls considerations Basic information Diagnosis [books.google.com]
The role of granulocyte colony-stimulating factor in which was used in the treatment of these patients remains to be established. [ncbi.nlm.nih.gov]
Practical, bulleted, highly templated text with easy-to-use features including flow charts and treatment boxes. [books.google.de]
PALLE HOLMSTRUP and MICHAEL GLICK, Treatment of periodontal disease in the immunodeficient patient, Periodontology 2000, 28, 1, (190-205), (2002). [joponline.org]
[…] or treatment delays. [academic.oup.com]
Prognosis
The prognosis of the patient is related both to the etiology of the neutropenia, as well as to the nadir of the neutrophil count. [mdedge.com]
Identification of a pathogenic variant may assist with prognosis, clinical management, familial screening, and genetic counseling. [mayocliniclabs.com]
Overview Historical Perspective Classification Pathophysiology Causes Differentiating Neutropenia from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications, and Prognosis Diagnosis History and Symptoms | Physical [wikidoc.org]
Hematology, also spelled haematology, is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. [1] [2] It involves treating diseases that affect the production of blood and its [en.wikipedia.org]
Prognosis The prognosis for mild or chronic neutropenia is excellent. Recovery from acute neutropenia depends on the severity of the patient's infection and the promptness of treatment. Resources Books Linker, Charles A. "Blood." [medical-dictionary.thefreedictionary.com]
Etiology
Etiologies There are various acquired and inherited etiologies that can account for fever and non-chemotherapy-induced neutropenia in children. In general, the acquired neutropenias are considered first because they are most common. [pedclerk.bsd.uchicago.edu]
Etiological form grouping congenital and acquired types and age groups, were investigated. Results: Forty-five cases were included, predominantly acquired forms (35, 81.4%). [pesquisa.bvsalud.org]
Etiology The etiologies of neutropenia in pediatrics are diverse. The most common causes of neutropenia are acquired. [learn.pediatrics.ubc.ca]
The prognosis of the patient is related both to the etiology of the neutropenia, as well as to the nadir of the neutrophil count. [mdedge.com]
The etiology of neutropenia can conceptually be viewed in two broad ways, by mechanism or etiologic category. The mechanisms that cause neutropenia are varied and not completely understood. [emedicine.medscape.com]
Epidemiology
“Changing epidemiology of infections in patients with neutropenia and cancer: emphasis on gram-positive and resistant bacteria”. [clinicaladvisor.com]
Overview Historical Perspective Classification Pathophysiology Causes Differentiating Neutropenia from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications, and Prognosis Diagnosis History and Symptoms | Physical [wikidoc.org]
Key words Chronic idiopathic neutropenia of adults (CINA) Epidemiology Leukocytes Perennial rhinitis This is a preview of subscription content, log in to check access. Preview Unable to display preview. Download preview PDF. [link.springer.com]
Forty-two articles described the epidemiology, pathobiology, and management of BEN and included 12 articles focusing on patients with BEN who developed decreased ANC during treatment with clozapine. [psychiatrist.com]
Epidemiology of chronic venous disease. Phlebology. 2008 ; 23 :103–111. Crossref Medline Google Scholar 4. Beebe-Dimmer JL, Pfeifer JR, Engle JS, Schottenfeld D. The epidemiology of chronic venous insufficiency and varicose veins. [ahajournals.org]
Pathophysiology
Overview Historical Perspective Classification Pathophysiology Causes Differentiating Neutropenia from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications, and Prognosis Diagnosis History and Symptoms | Physical [wikidoc.org]
Pathophysiology The pathophysiology of CINA is not well defined. This is partly a reflection of the heterogeneity of the disorder. Available information is discussed briefly below. [cancertherapyadvisor.com]
Provides details on pathophysiology, mutations and management of different types of congenital and acquired neutropenia.) Bodey, GP, Buckley, M, Sathe, YS. [clinicaladvisor.com]
The exact pathophysiology of lamotrigine-induced neutropenia is unknown (4). A literature review produced several reports of lamotrigine-induced neutropenia that reversed upon drug discontinuation (57). [ww1.cpa-apc.org]
[ edit ] The pathophysiology of neutropenia can be divided into congenital and acquired. [en.wikipedia.org]
Prevention
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]
Overuse of preventive regimens contributes to the development of treatment resistant strains of microbes. The preferred preventive drugs are an oral fluoroquinolone for bacteria and an oral triazole for fungi. [asco.org]
[…] and early detection of cervical cancer: American Cancer Society, American Society for Colposcopy and Cervical Pathology, and American Society for Clinical Pathology screening guidelines for the prevention and early detection of cervical cancer. [nccn.org]
How can you prevent infections? Take care of your skin Wash your hands often. You can use soap and water or an alcohol-based hand sanitizer. [northshore.org]
| Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies v t e Pathology : hematology, myeloid hematologic disease (primarily D50-D77, 280-289 ) RBCs / hemoglobinopathy + Polycythemia - Macrocytosis - Anemia Nutritional [wikidoc.org]