LOTUSHOSPITALNASHIK https://www.lotushaematology.com

Newsletter Vol 10 issue 2, Aug 2020

Dear friends and seniors,

I hope you all are fine and taking precautions. We all are adapting to the changing practices in the current scenario. This proves that “Change is the only Constant

I am dedicating this newsletter to my senior, an exceptional, honest, caring and above all a great human and physician late DrChudamanPatil sir from Dhule. Since I started my practice in 2008 he has been one of those physicians with whom I have had many conversations regarding patient care and throughout that journey he has taught me something new each time.The case I am discussing today was referred by him.

Mr BP is a 46 year old male who was referred to me 8 months ago with history of recurrent fever, leukocytosis and mild anaemia. On presentation he had fever for 14 days with mild bodyache, occasional chills, burning micturition. His Hb was 9.8, wbc was 27,600 with 82%neutrophils and platelet count was 5.14 lakhs. CRP was raised and ESR was 72 mm at end of 1 hour. 

His past history is really interesting!

He was first diagnosed with pulmonary Koch’s in 2006 when he fully recovered. He also had hospital visits almost twice yearly sine the time he remembers! His father was a government servant and they were in new city or town every 3 years and hence his treatment was overseen by many doctors. 

After 2006 he had Koch’s twice and each time he had full course of AKT. He always had a maculopapular skin rash with many medications.  He landed up with late DrPatil with PUO which was treated as bacterial infection. He recently went to Pune for PUO and was diagnosed with Fungal pneumonia (Aspergilosis) 3 months prior to his first visit to Lotus Hospital.

He had no family history of recurrent infections and he was born out of a non consanguineous marriage. He had atleast 14-15 hospital files with him and according to his wife this was only the tip of iceberg? His history was obviously suggestive of some underlying immune disorder. The source of infection when he first came to me was urinary tract infection ?prostatic infection. 

Workup:I thought of immunodeficiency even though his age was 46 years! The workup can be divided in 2 parts (basic and advanced)

His Immunoglobulin levels (IgG, IgM, IgA and even IgE) were very low. This is a very easy and cost effective test to diagnose immunodeficiency.Immunoglobulin deficiency can be congenital or acquired.

Common acquired causes are: Lymphoma, Myeloma, Chronic Lymphocytic leukemia etc.

Diagnosis: Congenital Hypogammaglobulinemia with recurrent infections.

We tested his 2 brothers who had normal immunoglobulin levels except for borderline low IgA levels.

Pathology: B lymphocyte mature into plasma cells in our bone marrow and they secrete immunoglobulins which are protective and inherent component of our immune defence. When they are low we get recurrent infections. 

Treatment:  we can give immunoglobulin infusion during serious infection. They help in reducing the morbidity and mortality. We gave 60mg of IVIG infusions to our patient after which his fever settled. IVIG infusions are short acting and they donot treat the disease permanently. He might need such infusions in future also. They act for a maximum of 1 month! It is also costly. 

Plan of future action: To prevent future infection he is now on once a week Bactrim DS prophylaxis.

Take home message: 

  1. Immunodeficiency disorders can also present in adults.
  2. If we diagnose them early we can prevent morbidity and mortality.
  3. Late diagnosis can also result in increased incidence of solid and hematological malignancies in such patients.
  4. Prophylactic antibiotics can help.
  5. Bone marrow transplantation is curative in such scenario.

I am always going to remember our senior late Dr.ChudamanPatil and really going to miss him for the rest of my life. May his soul rest in peace.   I am sure he must be helping someone wherever he is.

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