MCID: HCL001
MIFTS: 43

Hcl-V malady

Rare diseases, Cancer diseases, Blood diseases categories

Aliases & Classifications for Hcl-V

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 61UMLS, 39NCIt, 56SNOMED-CT, 27ICD9CM, 33MeSH, 26ICD10 via Orphanet, 62UMLS via Orphanet, 34MESH via Orphanet, 25ICD10
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Aliases & Descriptions for Hcl-V:

Name: Hcl-V 8 48
Hairy Cell Leukemia 8 42 10 48 61
Hairy Cell Leukemia Variant 8 48 61
Leukemic Reticuloendotheliosis 42 48
Hcl 42 48
 
Prolymphocytic Variant of Hairy Cell Leukemia 48
Leukemic Reticuloendotheliosis Variant 48
Prolymphocytic Variant of Hcl 48
Hairy Cell Leukaemia Variant 8
Leukemia Hairy Cell 44


Classifications:



Characteristics (Orphanet epidemiological data):

48
hairy cell leukemia variant:
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (United States); Age of onset: Adult; Age of death: adult
hairy cell leukemia:
Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe),1-9/1000000 (United States); Age of onset: Adult


External Ids:

Disease Ontology8 DOID:713, DOID:285
NCIt39 C7401, C7402
ICD9CM27 202.4
MeSH33 D007943
Orphanet48 300878, 58017
ICD10 via Orphanet26 C91.4
UMLS via Orphanet62 C0349633, C0023443
MESH via Orphanet34 D007943
ICD1025 C91.4

Summaries for Hcl-V

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NIH Rare Diseases:42 Hairy cell leukemia is a rare, slow-growing cancer of the blood in which the bone marrow makes too many b cells (lymphocytes), a type of white blood cell that fights infection. the condition is named after these excess b cells which look 'hairy' under a microscope. as the number of leukemia cells increases, fewer healthy white blood cells, red blood cells and platelets are produced. the underlying cause of this condition is unknown. while there is no cure, treatment can lead to remission which can last for years.   last updated: 4/3/2012

MalaCards based summary: Hcl-V, also known as hairy cell leukemia, is related to chronic lymphocytic leukemia and intrapelvic lymph node leukemic reticuloendotheliosis, and has symptoms including splenomegaly, leukemia and leukocytosis. An important gene associated with Hcl-V is BRAF (v-raf murine sarcoma viral oncogene homolog B). The drugs interferon alfa-2a and interferon alfa-2b and the compounds alanine and fatty acid have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, bone and b cells.

Related Diseases for Hcl-V

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Diseases related to Hcl-V via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 156)
idRelated DiseaseScoreTop Affiliating Genes
1chronic lymphocytic leukemia10.7
2intrapelvic lymph node leukemic reticuloendotheliosis10.7
3b-cell lymphomas10.6
4prolymphocytic leukemia10.5
5sarcoma10.5
6vasculitis10.5
7polycythemia vera10.5
8polycythemia10.5
9hodgkin lymphoma10.5
10splenic manifestation of hairy cell leukemia10.5
11hairy cell leukemia of spleen10.5
12splenic marginal zone lymphoma10.5
13gilles de la tourette syndrome10.4
14hyperparathyroidism10.4
15hemolytic anemia10.4
16mantle cell lymphoma10.4
17myelofibrosis10.4
18autoimmune hemolytic anemia10.4
19meningitis10.4
20myeloma10.4
21splenomegaly10.4
22aplastic anemia10.3
23lymphoid leukemia10.3
24marginal zone b-cell lymphoma10.3
25polyclonal hypergammaglobulinemia10.3
26macroglobulinemia10.3
27sporotrichosis10.3
28adenocarcinoma10.3
29dendritic cell sarcoma10.3
30guillain-barre syndrome10.3
31retinitis10.3
32sideroblastic anemia10.3
33acute febrile neutrophilic dermatosis10.3
34essential thrombocythemia10.3
35large granular lymphocyte leukemia10.3
36mycobacterium kansasii10.3
37refractory hairy cell leukemia10.3
38myeloid leukemia10.3
39prostatitis10.3
40attention deficit-hyperactivity disorder10.3
41pol iii-related leukodystrophies10.3
42myocardial infarction10.2
43porphyria cutanea tarda10.2
44melkersson-rosenthal syndrome10.2
45aspergillosis10.2
46lung cancer10.2
47bacteremia10.2
48renal cell carcinoma10.2
49glomerulonephritis10.2
50hematopoietic stem cell transplantation10.2

Graphical network of the top 20 diseases related to Hcl-V:



Diseases related to hcl-v

Symptoms for Hcl-V

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Symptoms:

 48 (show all 25)
  • acute abdominal pain/colic
  • splenomegaly
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • bone marrow/medullar infiltration
  • hyperleukocytosis/leukocytosis
  • myeloproliferative syndrome/chronic leukemia
  • asthenia/fatigue/weakness
  • anaemia
  • hyperlymphocytosis
  • cutaneous rash
  • macules
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • abnormal hepatic enzymes/transaminases
  • heart/cardiac failure
  • vascularitis/vasculitides/arteritis
  • lymphadenopathy/polyadenopathies
  • renal failure
  • bone marrow failure/pancytopenia
  • hemolytic anemia
  • monocytes/macrophages anomalies
  • hemorrhage/hemorrhagic syndrome/excessive/long-lasting bleeding
  • neoplasms/tumors
  • lung/bronchopulmonary neoplasm/tumor/carcinoma/cancer
  • prostate/prostatic neoplasm/tumor/carcinoma/cancer

HPO human phenotypes related to Hcl-V:

(show all 20)
id Description Frequency HPO Source Accession
1 splenomegaly hallmark (90%) HP:0001744
2 leukemia hallmark (90%) HP:0001909
3 leukocytosis hallmark (90%) HP:0001974
4 abdominal pain hallmark (90%) HP:0002027
5 respiratory insufficiency hallmark (90%) HP:0002093
6 abnormality of immune system physiology hallmark (90%) HP:0010978
7 lymphocytosis typical (50%) HP:0100827
8 renal insufficiency occasional (7.5%) HP:0000083
9 skin rash occasional (7.5%) HP:0000988
10 hypermelanotic macule occasional (7.5%) HP:0001034
11 congestive heart failure occasional (7.5%) HP:0001635
12 hemolytic anemia occasional (7.5%) HP:0001878
13 abnormality of coagulation occasional (7.5%) HP:0001928
14 vasculitis occasional (7.5%) HP:0002633
15 lymphadenopathy occasional (7.5%) HP:0002716
16 elevated hepatic transaminases occasional (7.5%) HP:0002910
17 abnormality of macrophages occasional (7.5%) HP:0004311
18 bone marrow hypocellularity occasional (7.5%) HP:0005528
19 neoplasm of the lung occasional (7.5%) HP:0100526
20 prostate neoplasm occasional (7.5%) HP:0100787

Drugs & Therapeutics for Hcl-V

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FDA approved drugs:

id Drug Name Active Ingredient(s)13 Pharmaceutical Company Approval Date
1
Intron A13 38 INTERFERON ALFA-2B Schering-Plough Approved December 1997/ Approved December 1995/ Approved March 1997
FDA Label: Intron A
Malady that Drug Treats: non-Hodgkin's lymphoma/ malignant melanoma / Hepatitis C
Indications and Usage:13 Hairy Cell Leukemia INTRON® A is indicated for the treatment of patients 18 years of; age or older with hairy cell leukemia.; Malignant Melanoma INTRON A is indicated as adjuvant to surgical treatment in; patients 18 years of age or older with malignant melanoma who are free of disease but; at high risk for systemic recurrence, within 56 days of surgery.; Follicular Lymphoma INTRON A is indicated for the initial treatment of clinically; aggressive (see Clinical Pharmacology) follicular Non-Hodgkin s Lymphoma in; conjunction with anthracycline-containing combination chemotherapy in patients 18; years of age or older. Efficacy of INTRON A therapy in patients with low-grade, lowtumor; burden follicular Non-Hodgkin s Lymphoma has not been demonstrated.; Condylomata Acuminata INTRON A is indicated for intralesional treatment of selected; patients 18 years of age or older with condylomata acuminata involving external; surfaces of the genital and perianal areas (see DOSAGE AND ADMINISTRATION).; The use of this product in adolescents has not been studied.; AIDS-Related Kaposi's Sarcoma INTRON A is indicated for the treatment of selected; patients 18 years of age or older with AIDS-Related Kaposi's Sarcoma. The likelihood; of response to INTRON A therapy is greater in patients who are without systemic symptoms, who have limited lymphadenopathy and who have a relatively intact immune; system as indicated by total CD4 count.; Chronic Hepatitis C INTRON A is indicated for the treatment of chronic hepatitis C in; patients 18 years of age or older with compensated liver disease who have a history of; blood or blood-product exposure and/or are HCV antibody positive. Studies in these; patients demonstrated that INTRON A therapy can produce clinically meaningful effects; on this disease, manifested by normalization of serum alanine aminotransferase (ALT); and reduction in liver necrosis and degeneration.; A liver biopsy should be performed to establish the diagnosis of chronic hepatitis.; Patients should be tested for the presence of antibody to HCV. Patients with other; causes of chronic hepatitis, including autoimmune hepatitis, should be excluded. Prior; to initiation of INTRON A therapy, the physician should establish that the patient has; compensated liver disease. The following patient entrance criteria for compensated liver; disease were used in the clinical studies and should be considered before INTRON A; treatment of patients with chronic hepatitis C:; No history of hepatic encephalopathy, variceal bleeding, ascites, or other; clinical signs of decompensation; Bilirubin Less than or equal to 2 mg/dL; Albumin Stable and within normal limits; Prothrombin Time Less than 3 seconds prolonged; WBC Greater than or equal to 3000/mm3; Platelets Greater than or equal to 70,000/mm3; Serum creatinine should be normal or near normal.; Prior to initiation of INTRON A therapy, CBC and platelet counts should be; evaluated in order to establish baselines for monitoring potential toxicity. These tests; should be repeated at Weeks 1 and 2 following initiation of INTRON A therapy, and; monthly thereafter. Serum ALT should be evaluated at approximately 3-month intervals; to assess response to treatment (see DOSAGE AND ADMINISTRATION).; Patients with preexisting thyroid abnormalities may be treated if thyroidstimulating; hormone (TSH) levels can be maintained in the normal range by medication.; TSH levels must be within normal limits upon initiation of INTRON A treatment and TSH; testing should be repeated at 3 and 6 months (see PRECAUTIONS, Laboratory; Tests).; INTRON A in combination with REBETOL® is indicated for the treatment of; chronic hepatitis C in patients 3 years of age and older with compensated liver disease; previously untreated with alpha interferon therapy and in patients 18 years of age and; older who have relapsed following alpha interferon therapy. See REBETOL prescribing; information for additional information. Chronic Hepatitis B INTRON A is indicated for the treatment of chronic hepatitis B in; patients 1 year of age or older with compensated liver disease. Patients who have been; serum HBsAg positive for at least 6 months and have evidence of HBV replication; (serum HBeAg positive) with elevated serum ALT are candidates for treatment. Studies; in these patients demonstrated that INTRON A therapy can produce virologic remission; of this disease (loss of serum HBeAg) and normalization of serum aminotransferases.; INTRON A therapy resulted in the loss of serum HBsAg in some responding patients.; Prior to initiation of INTRON A therapy, it is recommended that a liver biopsy be; performed to establish the presence of chronic hepatitis and the extent of liver damage.; The physician should establish that the patient has compensated liver disease. The; following patient entrance criteria for compensated liver disease were used in the; clinical studies and should be considered before INTRON A treatment of patients with; chronic hepatitis B:; No history of hepatic encephalopathy, variceal bleeding, ascites, or other; signs of clinical decompensation; Bilirubin Normal; Albumin Stable and within normal limits; Prothrombin Time Adults less than 3 seconds prolonged; Pediatrics less than or equal to 2 seconds prolonged; WBC Greater than or equal to 4000/mm3; Platelets Adults greater than or equal to 100,000/mm3; Pediatrics greater than or equal to 150,000/mm3; Patients with causes of chronic hepatitis other than chronic hepatitis B or chronic; hepatitis C should not be treated with INTRON A. CBC and platelet counts should be; evaluated prior to initiation of INTRON A therapy in order to establish baselines for; monitoring potential toxicity. These tests should be repeated at treatment Weeks 1, 2,; 4, 8, 12, and 16. Liver function tests, including serum ALT, albumin, and bilirubin,; should be evaluated at treatment Weeks 1, 2, 4, 8, 12, and 16. HBeAg, HBsAg, and; ALT should be evaluated at the end of therapy, as well as 3- and 6-months posttherapy,; since patients may become virologic responders during the 6-month period; following the end of treatment. In clinical studies in adults, 39% (15/38) of responding; patients lost HBeAg 1 to 6 months following the end of INTRON A therapy. Of; responding patients who lost HBsAg, 58% (7/12) did so 1 to 6 months post-treatment.; A transient increase in ALT greater than or equal to 2 times baseline value (flare); can occur during INTRON A therapy for chronic hepatitis B. In clinical trials in adults; and pediatrics, this flare generally occurred 8 to 12 weeks after initiation of therapy and; was more frequent in responders (adults 63%, 24/38; pediatrics 59%, 10/17) than in; nonresponders (adults 27%, 13/48; pediatrics 35%, 19/55). However, in adults and; pediatrics, elevations in bilirubin greater than or equal to 3 mg/dL (greater than or equal; to 2 times ULN) occurred infrequently (adults 2%, 2/86; pediatrics 3%, 2/72) during; therapy. When ALT flare occurs, in general, INTRON A therapy should be continued unless signs and symptoms of liver failure are observed. During ALT flare, clinical; symptomatology and liver function tests including ALT, prothrombin time, alkaline; phosphatase, albumin, and bilirubin, should be monitored at approximately 2-week; intervals (see WARNINGS).
DrugBank Targets:11 1. Interferon alpha/beta receptor 2; 2. Interferon alpha/beta receptor 1
Mechanism of Action:13 
Target: intracellular oncogene expression, natural killer and cytotoxic T-cells, microphage, cytokine production
Action: stimulation, induction (unspecified effects on oncogene expression)
FDA: -

Drug clinical trials:

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Genetic Tests for Hcl-V

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Anatomical Context for Hcl-V

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MalaCards organs/tissues related to Hcl-V:

31
Bone marrow, Bone, B cells, Lung, Prostate, Heart, Skin, Monocytes

Animal Models for Hcl-V or affiliated genes

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Publications for Hcl-V

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Articles related to Hcl-V:

idTitleAuthorsYear
1
Efficient vibrational and translational excitations of a solid metal surface: State-to-state time-of-flight measurements of HCl(v=2,J=1) scattering from Au(111). (19063576)
2008
2
Optical control of ground-state atomic orbital alignment: Cl(2P3/2) atoms from HCl(v=2,J=1) photodissociation. (17935395)
2007
3
Complete remission of hairy cell leukemia variant (HCL-v) complicated by red cell aplasia post treatment with rituximab. (16266917)
2005
4
Proliferative Hairy Cell Leukaemia (HCL-v) Resistant to Alpha-Interferon: Clinical, Diagnostic and In-Vitro Cellular Characteristics. (20394559)
1990

Variations for Hcl-V

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Expression for genes affiliated with Hcl-V

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Search GEO for disease gene expression data for Hcl-V.

Pathways for genes affiliated with Hcl-V

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Compounds for genes affiliated with Hcl-V

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44Novoseek
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Compounds related to Hcl-V according to GeneCards Suite gene sharing:

idCompoundScoreTop Affiliating Genes
1alanine449.3NDUFAB1, BRAF
2fatty acid449.2NDUFAB1, BRAF
3aspartate449.0NDUFAB1, BRAF

GO Terms for genes affiliated with Hcl-V

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Molecular functions related to Hcl-V according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1calcium ion bindingGO:00055099.3NDUFAB1, BRAF

Sources for Hcl-V

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2CDC
12ExPASy
13FDA
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet