Mar 5, 2015
Newsletter Vol. 6 Issue 1 , Feb.2015
Dear friends and colleagues,
I hope this edition of our newsletter finds you in pink of health. Cricket world cup is in full swing! conference season is over and exam fever is in the air. I have a very interesting case for you this month which hopefully will lead to rationale management of cases of PUO or recurrent fever.
23 yr old male was referred for leukocytosis and fever. When I saw him, he had bodyache. No palpable lymphadenopathy or hepatosplenomegaly. Signs of left sided pneumonitis was present.
Investigations: PBS: neutrophilia with toxic granulation (suggesting bacterial infection). No blasts or abnormal lymphoid cells. No haemoparasites were seen. ESR was 78, CRP was 9 times the upper limit.
Impression: fever due to bacterial infection causing neutrophilia. So what is unusual about this case??
Past history: patient had recurrent URTI and 2 episodes of LRTI till 5 years of age. He had documented Koch’s at 6 yrs. He had brought atleast 17 medical record files of various doctors (some of them were at home he said!!) He had been admitted atleast 27 times in his last 23 years and numerous antibiotic course as an outpatient.
Considering this exhilarating history any one would feel “something fishy” going on here! I decided to rule out immune deficiency state. Further work up revealed that his had no demonstrable immunoglobulin levels (IgG,IgM and IgA). His IgE levels were also low. CD4 and CD8 ration was slightly altered. HIV was negative and BSL was normal.
Diagnosis: Common variable Immunodeficiency or X-Linked agammaglobulinemia (no definite family history for latter diagnosis).
In above condition B lymphocytes donot function adequately and hence antibody production by plasma cells is very low leading to immunodeficiency and recurrent infections.
There are many types of congenital immunodeficiency diseases and syndromes and almost all are genetically determined.
How do I treat our patient now?
Blood culture is of importance in such cases. Atleast 20ml blood should be sent for culture. In this case the culture was negative as he received 4 days was IV antibiotics. He developed serious pneumonia. Bronchoalveolar lavage did not reveal any organisms. AFB was negative. Fungal culture was negative. He was on BIPAP for 2 days. He was given IVIg infusion and then he recovered 4 days later. He is at home now, but he will come again for fever any time soon as IVIg infusion is temporary treatment!
Immunodeficiency state can be seen from neonates till adulthood also. We need to remember !
Is there a permanent cure for our patient?
Stem cell (bone marrow) transplantation is the only curative treatment for congenital immune deficiency diseases. This procedure can be difficult as they are at high risk of serious infections during and after the transplant.
In whom should we suspect immunodeficiency state?
- Recurrent fever with 2 admissions (genuine!) to hospital in a year.
- Recurrent “boils” or folliculitis.
- Non-healing wounds.
- Episode of documented serious fungal infection.
- Patients with immune thrombocytopenia.
- Patients with persistent “high wbc” count or inappropriately “low wbc”
- Recurrent oral ulcers with no obvious reasons.
- Patients with Koch’s or other infection not responding to regular medications.
- We need to rule out congenital and acquired conditions and then treat such patients.
We have started a new unit called “Lotus institute Of Stem Cell Therapy and Regenerative Medicine” where we use autologous stem cells and platelet rich plasma, mesenchymal stem cells and other replica chopard la strada watches immune modulatory cells to treat morbid diseases which have not responded to routine medical and surgical procedures. Currently we are treating following diseases:
- Non-healing ulcers (diabetic, pressure sores etc)
- Non-healing fractures.
- Oral sub mucous fibrosis
- Osteo arthritis (PRP therapy) for symptomatic relief.
- Tendon repairs and injuries (with help of orthopaedic surgeon)
- We are not offering this therapy for neurological diseases at present as there is no concrete evidence for its use.
The cost for therapy is atleast 50% lower than other metros as we process the samples in house.
I hope you would refer your patients with above diseases and make their life easier!