SUMMARY OF THE CLINICAL HISTORY OF A PATIENT DIAGNOSTICATED OF IDIOPATHIC PULMONARY HEMOSIDEROSIS (IPH)
Male patient, aged 30, caucasian, 179 cm, 75 kg.
At the age of 4, he started with an important hypochromic anemia (4 gr of Hemoglobin), without any aparent cause, being the sole symptoms mucocutaneous paleness and weakness. For a year and a half, the disease was treated as a gastric ulcer, eventhough there were no hematic losses detected in dejections.
The diagnosis of Idiopathic Pulmonary Hemosiderosis was made during an episode of bronchial cold, in which chest X-ray showed bronchopneumonic infiltration that appeared and disappeared in different parts of the lungs. He didn´t suffered hemoptysis, but making him cough (he then was 5 ½ years old) it was possible to obtain some quantity of hemoptoic sputum that studied by pathologists showed the presence of hemosiderin-containing macrophages.
He was then studied in La Paz Hospital, in Madrid (Spain), making sensibilization test to a great amount of substances, including milk, with negative results, and studying his inmunological status, that was considered normal. Since then, his evolution has consisted in recurrent crisis with a frequency of once or twice a year.
These episodes usually consisted in light general weakness with symptoms of pharyngitis or cold, and mucocutaneous paleness; more rarely, important general affection and fever, that lasted 3-4 days. The chest X-rays showed typical bronchopneumonic images, and in blood analysis appeared severe hypochromic anemia, high reticulocyts-counf and hiperbilirubinemia. He had hemoptoic sputum, that was difficult to get, but not hemoptysis.
He was treated with high doses of corticoids, a milk free diet, and between episodes, with iron to reduce anemia. Corticoid therapy produced important secundary effects, that disappeared when it was discontinued.
From 12 to 16 years there was a complete remission of the process, during which periodic control were normal.
The crisis reappeared at 17 with the same symptoms and characteristics than before, again with a frequency of 1 ½ per year. Some of the crisis seemed to have a direct relation with stressed situations as infections, strong emotions or exercise (one of them developed after an effort test).
From this period, the treatment was systematized, consisting during crisis in 40- 60 mg per day of Prednisone for a 4 weeks period, then reducing dose to 30 mg per day during 2 weeks and finally 15 mg per day for another 2 weeks, to reach the intercrisis periods with 5 or 7.5 mg per day. For the last years, he has been taking regularly 5 mg/day. From July 1999 instead of 5 mg/day, I am taking 10 mg every two days.
During a several month lasting episode in 1977, he was treated with Mercaptopurin, with unsatisfactory results.
He has passed successive respiratory functional test with low results but in the rank of normality, and also has a normal osseal densitometry. Due to a great amount of X-ray exposures that he suffered during the initial period of the disease, in the last years only a few chest X-rays were made, so there is no continuos report. In the last one, made very recently, there is a micronudular image in both lungs.
The recurring episodes in the lasts years have occurred on Ago-94, Ago-96, Dec-96, Ago-97, Mar-98, and the last one on May-99.
Recently (May 1999), he has had a crisis with general weakness and hemoptoic sputums (three sputums during a week). The results of the laboratory test were as follows:
Before crisis During crisis
HCT 48.1 42.7
HB 16.1 15.3
FE 83µg/dl 43 µg/dl
FERI 150 ng/ml 93 ng/ml
RETIC 1.6% 1.8%
BILIRUBINE 1.26 mg/dl 1.88 mg/dl
The TAC of the chest revealed important diffuse interstitial affectation with a micronodular image and areas of alveolitis. In the left superior lobe, there is an alveolar image related to fresh bloody.
He has received the standard treatment above mentioned, from the start of the crisis (beginning with 60 mg/day of Metilprednisolona). The recovery of general weakness has been made quickly, just one week to feel better and improved, and without more hemoptoic sputum but the ones from the beginning.
Others explorations made recently, during the beginning of treatment, are reported:
CONCLUSIONS AND REQUIREMENTS
The patient is already an adult man. There are few information about this illness in adult patients. Thus, there are very few bibliography related to that, and it is difficult to find a hospital that may have this kind of patients. For that reason, I request of those who have experience of IPH in adults to report me theirs knowledge about some matters:
- Regarding to treatment, I wish to know opinions about the treatment that he is receiving, either in the crisis or between them. Also I wish to know others possible basics or coadjuvants treatments that had been useful in others patients, and they could be in this case.
- Other important matter is to make evident the reasons that starts the relapses. In this patient the stressed conditions have influenced on it, greatly, but there could exist others causes. What technics, procedures, biologic analysis,.... are able to discover some other causes ?.
- Finally, I wish to know impressions about the future prognostic of the illness and pulmonary function in this concrete case.
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