ICD-10-CM Diagnosis Codes1
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1 ICD-10-CM Diagnosis Codes G61 Chronic inflammatory demyelinating polyneuritis G61.81 Chronic inflammatory demyelinating polyneuritis Chronic inflammatory demyelinating polyneuropathy Chronic inflammatory demyelinating polyradiculoneuropathy Polyneuropathy (multiple nerve disorder) Polyneuropathy, chronic inflammatory demyelinating Polyradiculoneuropathy, chronic inflammatory demyelinating Polyradiculoneuropathy, inflammatory demyelinating D80 Immunodeficiency with predominantly antibody defects D80.0* Hereditary hypogammaglobulinemia Autosomal recessive agammaglobulinemia (Swiss type) X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency) D80.1 Nonfamilial hypogammaglobulinemia Agammaglobulinemia with immunoglobulin-bearing B-lymphocytes Common variable agammaglobulinemia [CVAgamma] Hypogammaglobulinemia NOS D80.2* Selective deficiency of immunoglobulin A [IgA] D80.3* Selective deficiency of immunoglobulin G [IgG] subclasses D80.4* Selective deficiency of immunoglobulin M [IgM] D80.5* Immunodeficiency with increased immunoglobulin M [IgM] D80.6* Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia D80.7* Transient hypogammaglobulinemia of infancy D80.8 Other immunodeficiencies with predominantly antibody defects Kappa light chain deficiency D80.9 Immunodeficiency with predominantly antibody defects, unspecified D81 Combined immunodeficiencies D81.0* Severe combined immunodeficiency [SCID] with reticular dysgenesis D81.1* Severe combined immunodeficiency [SCID] with low T- and B-cell numbers D81.2* Severe combined immunodeficiency [SCID] with low or normal B-cell numbers D81.4 Nezelof’s syndrome D81.5* Purine nucleoside phosphorylase [PNP] deficiency D81.6* Major histocompatibility complex class I deficiency Bare lymphocyte syndrome © 2020 CSL Behring LLC. The product information presented on this site is intended for US residents only. D81 Combined immunodeficiencies D81.7* Major histocompatibility complex class II deficiency D81.89* Other combined immunodeficiencies D81.9* Combined immunodeficiency, unspecified Severe combined immunodeficiency disorder [SCID] NOS Immunodeficiency associated with other major defects D82 Excludes: ataxia telangiectasia [Louis-Bar] (G11.3) D82.0* Wiskott-Aldrich syndrome Immunodeficiency with thrombocytopenia and eczema D82.1* Di George’s syndrome Pharyngeal pouch syndrome Thymic alymphoplasia Thymic aplasia or hypoplasia with immunodeficiency D82.2 Immunodeficiency with short-limbed stature D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus X-linked lymphoproliferative disease D82.4* Hyperimmunoglobulin E [IgE] syndrome D82.8 Immunodeficiency associated with other specified major defects D82.9 Immunodeficiency associated with major defect, unspecified D83 Common variable immunodeficiency D83.0* Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function D83.1* Common variable immunodeficiency with predominant immunoregulatory T-cell disorders D83.2* Common variable immunodeficiency with autoantibodies to B- or T-cells D83.8* Other common variable immunodeficiencies D83.9* Common variable immunodeficiency, unspecified G11 Hereditary Ataxia G11.3* Cerebellar ataxia with defective DNA repair Ataxia telangiectasia *Medicare Part B–approved diagnosis codes for treatment with Hizentra in the home. All other diagnoses may qualify for coverage under Medicare Part D plans. ICD=International Classification of Diseases Reference: 1. National Center for Health Statistics (US Department of Health and Human Services). International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM). 2020 Codes Tables and Index. https://www.cdc.gov/nchs/icd/icd10cm.htm. Accessed January 22, 2020. © 2020 CSL Behring LLC. The product information presented on this site is intended for US residents only. HIGHLIGHTS OF PRESCRIBING INFORMATION Administration (2.3) These highlights do not include all the information needed to use HIZENTRA • PI: Administer at regular intervals from daily up to every two weeks. safely and effectively. See full prescribing information for HIZENTRA. • CIDP: Administer weekly. Infusion sites – Up to 8 infusion sites are allowed simultaneously, with at least 2 inches HIZENTRA, Immune Globulin Subcutaneous (Human), 20% Liquid • between sites. Initial U.S. Approval: 2010 Administration in PI WARNING: THROMBOSIS st See full prescribing information for complete boxed warning. Infusion Parameters* 1 Infusion Subsequent Infusions Volume (mL/site) ≤15 ≤25 • Thrombosis may occur with immune globulin products, including HIZENTRA. Risk factors may include: advanced age, prolonged Rate (mL/hr/site) ≤15 ≤25 immobilization, hypercoagulable conditions, history of venous or *As tolerated arterial thrombosis, use of estrogens, indwelling vascular catheters, Administration in CIDP hyperviscosity, and cardiovascular risk factors. Infusion Parameters* 1st Infusion Subsequent Infusions • For patients at risk of thrombosis, administer HIZENTRA at the minimum Volume (mL/site) ≤20 ≤50 dose and infusion rate practicable. Ensure adequate hydration in patients Rate (mL/hr/site) ≤20 ≤50 before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity. * As tolerated ----------------------------DOSAGE FORMS AND STRENGTHS------------------------------ 0.2 g per mL (20%) protein solution for subcutaneous infusion available in a single-use ------------------------------------INDICATIONS AND USAGE--------------------------------- prefilled syringe (5 mL,10 mL, and 20 mL) or tamper-evident vial (5, 10, 20 and 50 mL). (3) HIZENTRA is an Immune Globulin Subcutaneous (Human) (IGSC), 20% Liquid indicated for the treatment of: -------------------------------------CONTRAINDICATIONS-------------------------------------- • Anaphylactic or severe systemic reaction to human immune globulin or inactive • Primary immunodeficiency (PI) in adults and pediatric patients 2 years of age and older. (1.1) ingredients of HIZENTRA, such as polysorbate 80. (4) • Maintenance therapy in adults with chronic inflammatory demyelinating polyneuropathy (CIDP). • Hyperprolinemia Type I or II (HIZENTRA contains stabilizer L-proline). (4) (1.2) • IgA-deficient patients with antibodies against IgA and a history of hypersensitivity. (4) ------------------------------DOSAGE AND ADMINISTRATION------------------------------- ---------------------------------WARNINGS AND PRECAUTIONS---------------------------- For subcutaneous infusion only. • IgA-deficient patients with anti-IgA antibodies are at greater risk of severe hypersensitivity and Dose (2.2) anaphylactic reactions. (5.1) PI • Thrombosis may occur following treatment with immune globulin products, including Before switching to HIZENTRA, obtain the patient’s serum IgG trough level to guide sub- sequent HIZENTRA. (5.2) dose adjustments. • Aseptic meningitis syndrome has been reported with IGIV or IGSC, including HIZENTRA • Weekly: Start HIZENTRA 1 week after last Immune Globulin Intravenous (Human) (IGIV) treatment. (5.3) infusion. • Monitor renal function, including blood urea nitrogen, serum creatinine, and urine output in Initial weekly dose = Previous IGIV dose (in grams) x 1.37 patients at risk of acute renal failure. (5.4) No. of weeks between IGIV doses • Monitor for clinical signs and symptoms of hemolysis. (5.5) • Biweekly (every 2 weeks): Start HIZENTRA 1 or 2 weeks after the last IGIV infusion or 1 week • Monitor for pulmonary adverse reactions (transfusion-related acute lung injury [TRALI]). (5.6) after the last weekly IGSC infusion. Administer twice the calculated weekly dose. • HIZENTRA is made from human blood and may contain infectious agents, e.g., viruses, the • Frequent dosing (2 to 7 times per week): Start HIZENTRA 1 week after the last IGIV or IGSC variant Creutzfeldt-Jakob disease (vCJD) agent and, theoretically, the Creutzfeldt- Jakob disease infusion. Divide the calculated weekly dose by the desired number of times per week. (CJD) agent. (5.7) • Adjust the dose based on clinical response and serum IgG trough levels. CIDP -----------------------------------ADVERSE REACTIONS---------------------------------------- • Initiate therapy with HIZENTRA 1 week after the last IGIV infusion. The most common adverse reactions observed in ≥5% of study subjects were local infusion site • Recommended subcutaneous dose is 0.2 g/kg (1 mL/kg) body weight (bw) per week. reactions, headache, diarrhea, fatigue, back pain, nausea, pain in extremity, cough, upper respiratory - In the clinical study after transitioning from IGIV to HIZENTRA, a dose of 0.4 g/kg (2 tract infection, rash, pruritus, vomiting, abdominal pain (upper), migraine, arthralgia, pain, fall and mL/kg) bw per week was also safe and effective to prevent CIDP relapse. nasopharyngitis. (6) • If CIDP symptoms worsen, consider re-initiating treatment with an IGIV approved for the To report SUSPECTED ADVERSE REACTIONS, contact CSL Behring Pharmacovigilance treatment of CIDP, while discontinuing HIZENTRA. at 1-866-915-6958 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. - If improvement and stabilization are observed during IGIV treatment, consider re- initiating HIZENTRA at 0.4 g/kg bw per week, while discontinuing IGIV. -----------------------------------DRUG INTERACTIONS--------------------------------------- - If CIDP symptoms worsen on 0.4 g/kg bw per week, consider re-initiating therapy with The passive transfer of antibodies may