2024 ICD-10-CM Diagnosis Code D69.42

Congenital and hereditary thrombocytopenia purpura

ICD-10-CM Code:
ICD-10 Code for:
Congenital and hereditary thrombocytopenia purpura
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Code Navigator:

Code Classification

  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
    • Coagulation defects, purpura and other hemorrhagic conditions
      • Purpura and other hemorrhagic conditions

D69.42 is a billable diagnosis code used to specify a medical diagnosis of congenital and hereditary thrombocytopenia purpura. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Amegakaryocytic thrombocytopenia
  • Amegakaryocytic thrombocytopenia
  • Amegakaryocytic thrombocytopenia
  • Amegakaryocytic thrombocytopenia with congenital malformation
  • Amegakaryocytic thrombocytopenia with congenital malformation
  • Autosomal dominant macrothrombocytopenia
  • Autosomal dominant thrombocytopenia with platelet secretion defect
  • Beta thalassemia X-linked thrombocytopenia syndrome
  • Bleeding diathesis due to thromboxane synthesis deficiency
  • Congenital amegakaryocytic thrombocytopenia
  • Congenital autosomal recessive small-platelet thrombocytopenia
  • Congenital dyserythropoietic anemia
  • Congenital thrombocytopenia
  • DIAPH1-related sensorineural hearing loss-thrombocytopenia syndrome
  • GATA binding protein 1 related thrombocytopenia with dyserythropoiesis
  • Giant platelet syndrome
  • Giant platelet syndrome
  • Hereditary thrombocytopenia with early-onset myelofibrosis
  • Hereditary thrombocytopenia with normal platelets
  • Hereditary thrombocytopenic disorder
  • Macrothrombocytopenia, lymphedema, developmental delay, facial dysmorphism, camptodactyly syndrome
  • Megakaryocytic thrombocytopenia
  • Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
  • MYH9 related disease
  • Platelet secretory disorder
  • Radioulnar synostosis with amegakaryocytic thrombocytopenia syndrome
  • Secondary thrombocytopenia
  • Severe autosomal recessive macrothrombocytopenia
  • Sex-linked thrombocytopenia
  • Thrombotic microangiopathy
  • Thrombotic thrombocytopenic purpura
  • Upshaw-Schulman syndrome
  • X-linked congenital dyserythropoietic anemia with thrombocytopenia
  • X-linked thrombocytopenia with normal platelets

Clinical Classification

Clinical Information

  • CDAN1 Gene|CDAN1|CDAN1|Congenital Dyserythropoietic Anemia, Type I Gene

    this gene may be involved in nuclear membrane maintenance.
  • CDAN1 wt Allele|CDA-I|CDA1|CDAI|Codanin Gene|Congenital Dyserythropoietic Anemia, Type I wt Allele|DLT|Discs Lost Homolog Gene|Discs Lost, Drosophila, Homolog of Gene|PRO1295|UNQ664/PRO1295

    human cdan1 wild-type allele is located in the vicinity of 15q15.2 and is approximately 14 kb in length. this allele, which encodes codanin-1 protein, may play a role in the maintenance of the nuclear envelope. mutation of the gene is associated with congenital dyserythropoietic anemia type i.
  • Congenital Dyserythropoietic Anemia

    a rare group of disorders that result in anemia that is caused by ineffective erythropoiesis, which is associated with multinuclear erythroblasts, and which may present in childhood. the most common mutations are in the cdan1 and sec23b genes.
  • Congenital Dyserythropoietic Anemia Type II|CDA II|CDAN2|HEMPAS|Hereditary Erythroblastic Multinuclearity with Positive Acidified-Serum Test|SEC23B-CDG

    an autosomal recessive subtype of congenital dyserythropoietic anemia caused by mutation(s) in the sec23b gene, encoding protein transport protein sec23b.
  • Congenital Dyserythropoietic Anemia Type IV|CDAN4

    an autosomal dominant sub-type of congenital dyserythropoietic anemia caused by mutation(s) in the klf1 gene, encoding krueppel-like factor 1.
  • SEC23B wt Allele|CDA-II|CDAII|CDAN2|Congenital Dyserythropoietic Anemia, Type II Gene|HEMPAS|RP11-379J5.1|Sec23 Homolog B (S. cerevisiae) wt Allele

    human sec23b wild-type allele is located in the vicinity of 20p11.23 and is approximately 54 kb in length. this allele, which encodes protein transport protein sec23b, is involved in the transport of vesicles from the endoplasmic reticulum to the golgi. mutation of the gene is associated with congenital dyserythropoietic anemia type ii.
  • Congenital Amegakaryocytic Thrombocytopenia

    a rare, autosomal recessive inherited disorder caused by mutation in the c-mpl gene. it is characterized by thrombocytopenia and absence of megakaryocytes. it presents with bleeding in the first month of life.

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Convert D69.42 to ICD-9-CM

  • ICD-9-CM Code: 287.33 - Cong/herid thromb purpra

Patient Education

Platelet Disorders

Platelets, also known as thrombocytes, are blood cells. They form in your bone marrow, a sponge-like tissue in your bones. Platelets play a major role in blood clotting. Normally, when one of your blood vessels is injured, you start to bleed. Your platelets will clot (clump together) to plug the hole in the blood vessel and stop the bleeding. You can have different problems with your platelets:

  • If your blood has a low number of platelets, it is called thrombocytopenia. This can put you at risk for mild to serious bleeding. The bleeding could be external or internal. There can be various causes. If the problem is mild, you may not need treatment. For more serious cases, you may need medicines or blood or platelet transfusions.
  • If your blood has too many platelets, you may have a higher risk of blood clots.
    • When the cause is unknown, this is called thrombocythemia. It is rare. You may not need treatment if there are no signs or symptoms. In other cases, people who have it may need treatment with medicines or procedures.
    • If another disease or condition is causing the high platelet count, it is thrombocytosis. The treatment and outlook for thrombocytosis depends on what is causing it.
  • Another possible problem is that your platelets do not work as they should. For example, in von Willebrand Disease, your platelets cannot stick together or cannot attach to blood vessel walls. This can cause excessive bleeding. There are different types of in von Willebrand Disease; treatment depends on which type you have.

NIH: National Heart, Lung, and Blood Institute

[Learn More in MedlinePlus]

Thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura is a rare disorder that causes blood clots (thrombi) to form in small blood vessels throughout the body. These clots can cause serious medical problems if they block vessels and restrict blood flow to organs such as the brain, kidneys, and heart. Complications resulting from these clots can include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal kidney function, abdominal pain, and heart problems.

Blood clots normally form to stop blood loss at the sites of blood vessel injury. In people with thrombotic thrombocytopenic purpura, clots develop even in the absence of apparent injury. Blood clots are formed from clumps of cells called platelets that circulate in the blood and assist with clotting. Because a large number of platelets are used to make clots in people with thrombotic thrombocytopenic purpura, fewer platelets are available in the bloodstream. A reduced level of circulating platelets is known as thrombocytopenia. Thrombocytopenia can lead to small areas of bleeding just under the surface of the skin, resulting in purplish spots called purpura.

This disorder also causes red blood cells to break down (undergo hemolysis) prematurely. As blood squeezes past clots within blood vessels, red blood cells can break apart. A condition called hemolytic anemia occurs when red blood cells are destroyed faster than the body can replace them. This type of anemia leads to paleness, yellowing of the eyes and skin (jaundice), fatigue, shortness of breath, and a rapid heart rate.

There are two major forms of thrombotic thrombocytopenic purpura, an acquired (noninherited) form and a familial (inherited) form. The acquired form usually appears in late childhood or adulthood. Affected individuals may have a single episode of signs and symptoms, or, more commonly, they may experience multiple recurrences over time. The familial form of this disorder is much rarer and typically appears in infancy or early childhood, although it can appear later in life. In people with the familial form, signs and symptoms often recur on a regular basis and may return during times of stress, such as during illness or pregnancy.

[Learn More in MedlinePlus]

X-linked thrombocytopenia

X-linked thrombocytopenia is a bleeding disorder that primarily affects males. This condition is characterized by a blood cell abnormality called thrombocytopenia, which is a shortage in the number of blood cells involved in clotting (platelets). Affected individuals often have abnormally small platelets as well, a condition called microthrombocytopenia. X-linked thrombocytopenia can cause individuals to bruise easily or have episodes of prolonged bleeding following minor trauma or even in the absence of injury (spontaneous bleeding). Some people with this condition experience spontaneous bleeding in the brain (cerebral hemorrhage), which can cause brain damage that can be life-threatening.

Some people with X-linked thrombocytopenia also have patches of red, irritated skin (eczema) or an increased susceptibility to infections. In severe cases, additional features can develop, such as cancer or autoimmune disorders, which occur when the immune system malfunctions and attacks the body's own tissues and organs. It is unclear, however, if people with these features have X-linked thrombocytopenia or a more severe disorder with similar signs and symptoms called Wiskott-Aldrich syndrome.

Some people have a mild form of the disorder called intermittent thrombocytopenia. These individuals have normal platelet production at times with episodes of thrombocytopenia.

[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.


[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.