D00-D89

Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

Chapter 3 of the 2026 ICD-10-CM (D50-D89) constitutes the primary classification for hematologic and immunologic pathology. This chapter encompasses a wide clinical spectrum, beginning with nutritional anemias (D50-D53) caused by iron, vitamin B12, or folate deficiencies. It expands into complex hemolytic anemias (D55-D59), including hereditary conditions like sickle-cell disease and thalassemias, as well as acquired autoimmune hemolytic anemias. Bone marrow failure syndromes (D60-D64), such as aplastic anemia and pure red cell aplasia, are detailed here alongside life-threatening coagulation defects (D65-D69) like hemophilia, von Willebrand disease, and disseminated intravascular coagulation (DIC). The chapter also addresses disorders of white blood cells and the spleen (D70-D77) and concludes with certain disorders involving the immune mechanism (D80-D89). These include primary immunodeficiency syndromes like common variable immunodeficiency (CVID) and severe combined immunodeficiency (SCID), as well as multisystemic granulomatous diseases like sarcoidosis. Diagnostic evaluation typically requires advanced hematopathology, flow cytometry, and genetic testing to differentiate between primary genetic etiologies and secondary reactive states.

Clinical Symptoms

  • Progressive fatigue and generalized weakness
  • Exertional dyspnea and shortness of breath
  • Pallor of the skin, conjunctiva, and nail beds
  • Easy bruising (ecchymosis) and pinpoint skin hemorrhages (petechiae)
  • Frequent, severe, or opportunistic infections
  • Recurrent epistaxis and mucosal bleeding
  • Jaundice (icterus) and dark-colored urine
  • Splenomegaly (enlarged spleen) and associated left upper quadrant fullness
  • Lymphadenopathy (enlarged lymph nodes)
  • Tachycardia and heart palpitations
  • Failure to thrive (in pediatric immune deficiency cases)
  • Paresthesia or glossitis (associated with B12 deficiency)
  • Vaso-occlusive pain crises (in sickle cell disorders)

Common Causes

  • Nutritional deficiencies (Iron, Vitamin B12, and Folate)
  • Genetic mutations (e.g., HBB gene in Sickle Cell/Thalassemia, F8/F9 in Hemophilia)
  • Autoimmune destruction of blood components (e.g., ITP, AIHA, and Pernicious Anemia)
  • Bone marrow suppression from radiation, chemotherapy, or environmental toxins (e.g., Benzene)
  • Congenital defects in immune cell maturation (e.g., BTK mutation in Agammaglobulinemia)
  • Chronic inflammatory states and chronic kidney disease causing decreased erythropoiesis
  • Viral triggers (e.g., Parvovirus B19, HIV, EBV, and Hepatitis viruses)
  • Secondary splenic dysfunction or post-surgical complications

Documentation & Coding Tips

Distinguish between iron deficiency anemia and anemia of chronic disease by documenting underlying etiology and laboratory markers such as ferritin and total iron binding capacity.

Example: Patient with Stage 4 Chronic Kidney Disease presents with worsening fatigue. Laboratory evaluation reveals Hemoglobin 9.2 g/dL, Ferritin 450 ng/mL, and Transferrin Saturation 18 percent. Assessment: Anemia in chronic kidney disease, stage 4, demonstrating iron sequestration and erythropoietin deficiency. Plan: Initiate erythropoiesis-stimulating agent therapy.

Billing Focus: Identify the underlying chronic condition, such as CKD stage or malignancy, to satisfy the linked code requirement for D63.1 or D63.0.

Specify the exact coagulation factor deficiency in hemorrhagic conditions to ensure precise code assignment within the D66-D68 range.

Example: Patient with established Hemophilia A (Factor VIII deficiency) presents with spontaneous hemarthrosis of the right knee. Coagulation profile shows Factor VIII level at 2 percent. Treatment involves administration of recombinant Factor VIII concentrate to achieve 50 percent activity level.

Billing Focus: Document laterality of the affected joint and the specific factor replaced to support high-intensity procedure codes.

Differentiate between secondary and primary neutropenia by documenting the suspected cause, such as chemotherapy-induced versus congenital cyclical neutropenia.

Example: Patient currently undergoing chemotherapy for breast cancer presents with Absolute Neutrophil Count of 450 per microliter. Patient is febrile at 101.5 F. Diagnosis: Neutropenic fever and drug-induced neutropenia. Admitted for IV antibiotic therapy and G-CSF support.

Billing Focus: Document the external cause code (T-code) for the chemotherapeutic agent to provide a complete billing narrative for drug-induced blood disorders.

When documenting immune deficiency, clearly state whether the condition is congenital or acquired and identify specific immunoglobulin deficiencies.

Example: Adult patient with recurrent sinopulmonary infections. Quantitative immunoglobulin testing shows IgG level of 300 mg/dL and low IgA. Diagnosis: Common variable immunodeficiency (CVID). Plan: Monthly intravenous immunoglobulin (IVIG) infusions.

Billing Focus: Specific laboratory findings (IgG levels) must be documented to support the medical necessity of long-term IVIG therapy.

Document the presence of pancytopenia versus isolated cytopenias and specify if the condition is due to myelodysplasia or bone marrow failure.

Example: Elderly patient with progressive shortness of breath. Bone marrow biopsy reveals hypocellular marrow with no evidence of malignancy. Diagnosis: Idiopathic aplastic anemia. Laboratory shows pancytopenia with Hgb 7.1, WBC 2.2, and Platelets 45,000.

Billing Focus: Use D61.3 for idiopathic aplastic anemia. If drug-induced, specific drug coding is required for optimal specificity.

Relevant CPT Codes

Related Diagnoses

Hierarchy