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Documentation and Coding: Coagulation Defects & Other Specified Hematological Disorders
March 20, 2023
Claims & Coding Physicians & Specialists

A quick reference guide to assist with accurate, complete documentation and coding that reflects the true nature of a patient’s current health status at the highest level of specificity. Per ICD-10 official guidelines for reporting and coding, “The importance of consistent, complete documentation in the medical record cannot be overemphasized. Without such documentation, accurate coding cannot be achieved.”  

Coagulation defects – also known as bleeding disorders and blood clotting disorders – are problems with your blood's ability to form clots. The defects can involve your blood vessels and the cells and proteins in your blood. Coagulation disorders are conditions that affect the blood’s clotting activities. Hemophilia, Von Willebrand disease, clotting factor deficiencies, hypercoagulable states, and deep venous thrombosis are all coagulation disorders. Hemophilia and Von Willebrand disease are among the best known.

Documentation & Coding Tips 

Documentation needs to be clear and detailed using the following terms to allow accurate ICD10 code selection.

  • Specificity: Include the cause of the disorder (hereditary, drug-induced) 
  • Treatment: Document treatment (medication, monitoring) and current response to treatment.   
  • Thrombocytosis & thrombocythemia are not interchangeable with thrombocytopenia.  
  • Thrombocytopenia is commonly seen in PMH/PL. To accept this condition there must be evidence that it’s still present (ex. current platelet count). Without current evidence of the disease, this code cannot be captured.  
  • Anticoagulant/anticoagulation and long-term coagulation are not interchangeable with coagulopathy. These are indicators that a patient is on a coagulation medication, and it could be for many different reasons.  
  • Polycythemia vera is not interchangeable with polycythemia.  
  • Examples of conditions that can cause a secondary hypercoagulable state are atrial fibrillation, malignancy, pregnancy, trauma, myeloproliferative disorders, and antiphospholipid antibody syndrome.

ICD 10 Code 

Description 

Notes 

C94.6 

Myelodysplastic disease, not elsewhere classified 

Myelodysplastic syndrome (bone marrow problem) 

D45 

Polycythemia vera 

Abnormal proliferation of all bone marrow elements, increased red cell mass, and total blood volume. The etiology is unknown, but it is frequently associated with splenomegaly, leukocytosis, and thrombocythemia. 

D47.xx 

Other neoplasms of uncertain behavior of lymphoid, hematopoietic, and related tissue 

Chronic problem with blood cell production 

D55.xx 

Anemia due to enzyme disorders 

Red blood cells produce enzymes (proteins) to help protect them. When these enzymes are missing due to gene mutation, the red blood cells become fragile and susceptible to early damage or destruction. 

D56.x 

Thalassemia 

Thalassemia’s are inherited disorders of the red blood cells caused by a genetic mutation or deletion of the genes needed for proper hemoglobin formation. 

D57.3 

Sickle-cell trait 

Heterozygous genetic makeup is characterized by one gene for normal hemoglobin and one for sickle-cell hemoglobin. The clinical disease is rarely present. 

D58.x 

Other hereditary hemolytic anemias 

Conditions under this category are genetic forms of hemolytic anemia but do not fall under the more specific categories for thalassemia and sickle-cell disorders. 

D64.xx 

Other anemias 

This category reports other types of anemia such as sideroblastic anemia, congenital dyserythropoietic anemia, and anemia due to antineoplastic chemotherapy. 

D65 

Disseminated intravascular coagulation (DIC) [defibrination syndrome] 

Bleeding problem with overactive clotting 

D68.xxx 

Other coagulation defects 

Note-Von Willebrand disease has had a code expansion (10/2022) 

D69.xx 

Purpura and other hemorrhagic conditions 

Purpura refers to hemorrhage, or extravasation of blood, into the tissues or organs. When this occurs on the skin, the condition appears as bruises and small red patches. Depending on the size it may also be referred to as petechiae or ecchymosis. Purpura may be associated with thrombocytopenia, a decrease in the number of platelets circulating in the blood, but it can also occur in a nonthrombocytopenic form. 

D75.82x 

Heparin induced thrombocytopenia (HIT) 

Use additional code, if applicable, for adverse effect of heparin (T45.515-) 

References:

Johns Hopkins Medicine on Coagulation Disorders

Updated CMS ICD-10 Coding Guidelines (PDF)

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