Blood Hematopoietic Disease: Central Diabetes Insipidus

• Disease which stride over Endocrine Department and Blood Internal Medicine Department.
• The cause is the abnormality in hypothalamic area or pituitary area.
• Symptoms are drying of the throat, polyposis, and excessive urination.
• Able to prevent development by early stage treatment.
• Examine urine amount or concentration of electrolyte.
• Treatment for primary disease has the priority.

Disease which strides over Endocrine Department and Blood Internal Medicine Department.

Central Diabetes Insipidus is the endocrine disease which major complaint is polyposis and excessive urination as the result of producing extremely thin urine excessively due to the shortage of antidiuretic hormone (ADH) and arginine vasopressin (AVP). At a glance, it is perceived as no relation to blood disease, but in fact, it is necessary to understand that both diseases are closely related with each other. Central Diabetes Insipidus is developed in relation to several blood diseases. Central Diabetes Insipidus is taken care of at Endocrine Department, and blood disease is taken care of at Blood Internal Medicine Department. So that there are quite lot of cases where cooperation of these departments is necessary.

The cause is the abnormality in hypothalamic area or pituitary area.

There are Nephrogenic Diabetes Insipidus and Central Diabetes Insipidus in diabetes insipidus disease. Central diabetes insipidus is developed by the loss of produced amount of antidiuretic hormone which involves in the adjustment of the amount of water in the body. Antidiuretic hormone is the produced at hypothalamic area, and pooled in posterior lobe of gland, then discharged into the blood. In case when disorder is developed at hypothalamic area or hypothalamic pituitary region (HPR) which relate to these process, it becomes a cause of Nephrogenic Diabetes Insipidus. In these diseases, there are embryonic tumor which is developed at hypothalamic area or hypothalamic pituitary region (HPR), Langerhans cell histiocytosis (LCH), or its affinity histiocytosis of Erdheim-Chester Disease (ECD), sarcoidosis or tuberculosis, and HPR Surgery Damage, Brain trauma such as skull base fracture, Aneurysm and obstruction of the cerebral artery, Encephalitis and meningitis, etc.There may be the case where the disease is diagnosed as Lymphocytic hypophysis at Endocrine System Department.

Other than the above, in the blood disease, Acute myeloid leukemia (AML), Bone marrow dysplasia syndrome (MDS), Chronic bone marrow mononuclear leukemia (CMMOL), Juvenile myeloid mononuclear leukemia (JMML) and Acute lymphocytic leukemia (ALL) are reported, as the disease which develops the Central Diabetes Insipidus by leukemia cell infiltrated in HPR.

Symptoms are drying of the throat, polyposis, and excessive urination.

Symptoms of Central Diabetes Insipidus are drying of the throat, polyposis, and excessive urination. As the variety of symptoms like, attack of fever, anemia or Liver and spleen swelling in Leukemia and other blood disorders, and eruption, bone lesion, fibers of the retroperitoneal and surrounding vessels in LCH or ECD, are appeared at other part of central nerve, what is of paramount importance is to keep an eye on the symptoms of diabetes insipidus creeped into the aforementioned variety of the symptoms.

Able to prevent development by early stage treatment.

Looking into the relationship between Central Diabetes Insipidus and LCH, LCH occur in few years later the onset of Central Diabetes Insipidus, and there is a case Central Diabetes Insipidus occur, at the same time of LCH onset, after LCH onset, or few years later. There is a case where assumed diagnosis is made as Lymphocytic hypophysis, so that while following progress for few years, it is diagnosed at last as LCH. Normally, established Central Diabetes Insipidus is considers as irreversible and hard to cure, but it is possible to stop the development of diabetes insipidus if primary disease is treated well at early development stage during the period of partially diabetes insipidus status.

Examine urine amount or concentration of electrolyte.

Differential diagnosis of diabetes insipidus includes the distinguish of whether central, or nephrogenic, or SIADH. Best method for the diagnosis is water deprivation test, which measures, periodically for 12 hours, the urine amount, concentration of electrolyte, and body weight. It confirms the low value with measuring the density of Serum vasopressin (arginine vasopressin =AVP、antidiuretic hormone= ADH）. Detail examination of HPR by Brain MRI Exam is of the essence. It becomes the confirmed diagnosis when disappearance of hot signal (pooled amount of antidiuretic hormone) at posterior pituitary gland in the T1 enhanced image. (fig.1)

Contrast tumor Shadow is recognized from the pituitary stalk to the hypothalamus by GD radiography in the diseases, like LCH, that form a mass. (fig.2) And for LCH or ECD, disorder is fixed by pathology diagnosis with conducting biopsy to the peripheral organ, even though pathological change of HPR is unable to conduct biopsy. Blood disease involvement can also be diagnosed by hemogram and myelogram. For the diagnosis of embryonic tumor, hCG-beta measurement of blood serum and cerebral spinal fluid is useful. For sarcoidosis, Ocular uveitis, enlarged bilateral hilar lymph node tumor, ACE in blood, and high value of Lysozyme are referenced. In case when definition of the disease can not be reached by any means even though disorder exists in HPR, it may become necessary to conducts pathological diagnosis by biopsy of HPR.

Treatment for primary disease has the priority.

Causative disease is prioritized in the treatment. In case of early stage of diabetes insipidus, there is a case where the disease is cured by causative disease treatment. The report says that the MDS caused diabetes insipidus is improved by bone-marrow transplant. For once established diabetes insipidus, desmopressin (DDAVP) is administered few times a day through nasal spray or nose drop. Oral medicine like mini phosphorus melt OD tablet (one-time dosage: 60-120µg) can be used.

Fig.1 Disappearance of high signal at posterior pituitary gland (arrowed) seen in T1 enhanced image (TR=400) of MRI means Central diabetes insipidus.

Fig.2 LCH4 case: Infiltrative shadow to HPR seen at A thru D.(High Signal can be seen from the pituitary stalk to the hypothalamus by GD radiography. In each case, Central diabetes insipidus is developed.)