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 Table of Contents  
LETTER TO THE EDITOR
Year : 2014  |  Volume : 19  |  Issue : 2  |  Page : 170-171

POEMS Syndrome: Response to queries


Department of Critical care medicine CMC, Vellore, Tamil Nadu, India

Date of Web Publication11-Aug-2014

Correspondence Address:
T Iadarling
CMC, Vellore, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9903.138451

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How to cite this article:
Iadarling T. POEMS Syndrome: Response to queries. J Mahatma Gandhi Inst Med Sci 2014;19:170-1

How to cite this URL:
Iadarling T. POEMS Syndrome: Response to queries. J Mahatma Gandhi Inst Med Sci [serial online] 2014 [cited 2020 Jul 6];19:170-1. Available from: http://www.jmgims.co.in/text.asp?2014/19/2/170/138451

Sir,

I would like to thank Dr.Lalit for interest in the article and would like to clarify certain things.

Going first to the first point that you pointed that megaloblastic anemia is being taken by mean corpuscular volume (MCV) >100, we would partly agree to it but for diagnosing it, however it includes: Anemia <13 g/dl in males and <12 g/dl in females, macrocytic red cells (MCV > 100 fl), hypersegmented neutrophils, with or without neurological disturbances and pancytopenia (of uncertain cause).Our patient had definitely three of the above which goes very well with the most common differential diagnosis megaloblastic anemia, especially in our Indian rural population, hence we were justified enough for a diagnosis. As for MCV of 100 fl in our case, it was a higher normal value, just at the margin between normal and abnormal values. The probability of cobalamin and folate deficiency being present with MCV being normal was seen in <25% from studies conducted. [1]

With anemia, hyper segmented neutrophils, neurological picture and skin changes; hence the diagnosis of megaloblastic anemia was reasonable enough. We could have done Vitamin B12 and folate levels for a definitive diagnosis, however in a rural setup where cost is a problem it could not be done. Since it's also mentioned that therapeutic trials of cobalamin are warranted when testing are in conflict with the diagnosis, hence treating the patient as a megaloblastic anemia was acceptable with the above picture he presented with.

From, the presentation of the patient, the diagnostic criteria taken into account, and the limitations in our diagnostic workup, answers the question of why he was taken initially as a case of megaloblastic anemia. Another point to clarify in your note Sir, he was a 57-year-old who was never healthy as you have mentioned, with his problems that were progressing due to underlying syndrome that was later diagnosed.

Secondly: The skin hyper pigmentation which our patient had could not be photographed as our patient's consent was not obtained due to personal reasons. However, the hyperpigmentation had primarily upper limb involvement. Hyper pigmentation of the skin occurs in many conditions. For our patient, it was initially thought to be due to megaloblastic anemia as common things come first. However, we feel it is due to the malignancy, making it one of the minor criteria. Hyper pigmentation has been documented in 40% of the patients with POEMS syndrome. [2]

Thirdly: Sir, we would like to agree with you that the background diagnosis of megaloblastic anemia in our patient could have been there. But there is another more prominent disease in him that have produced progression of the primary symptoms, and other clinical signs which were looked into making megaloblastic anemia picture masking the other disease.

Fourth point: Yes we would like to clear the statement that we have made in the article. Both the serum protein electrophoresis (SPEP) and immunofixation study were done which had confirmed the presence of the lambda restricted M protein. It would have been a much better picture of the evidence of monoclonal plasma cell proliferation abnormality if the urine protein electrophoresis had been done. But due to financial constraint, it was not able to be carried out.

Fifth point: Of why multiple myeloma seemed unlikely, as you have mention there is the absence of organ or tissue involvement elevated serum calcium, renal insufficiency, anemia and lytic bone injury, bone marrow done was not also not suggestive of >10% of plasma cells.

As for the serum electrophoresis, there should be the presence of the M protein hence mention wrongly.

As for polyneuropathy, it is uncommon in the classical multiple myeloma, and when present it is usually due to amyloidosis. As there were no features suggestive of amyloidosis, hence polyneuropathy in myeloma seemed unlikely and the most common neurological complication seen in multiple myeloma is radiculopathy.

Sixth point: Sir, we would like to thank you again for pointing out that the statement is not clear regarding Waldenstorm macroglobulinemia. To make the point more clear as to why the case does not seemed to be Waldenstorm macroglobulinemia are:

  1. Absence of the monoclonal immunoglobin M protein in the SPEP.
  2. Bone marrow and lymph node biopsy did not show abnormal lymphoplasmacytic cells.


Absence of these features makes diagnosis of Waldenstorm macroglobulinemia unlikely. Organomegaly is seen in a small percentage of the cases. [3]

Seventh point: I would also like to thank you again Sir, for pointing out that our patient had another additional minor criteria, thrombocytosis (platelets >450,000) supporting the diagnosis of POEMS.

Lastly, Sir, as for refraining the sentence that all cases polyneuropathy with megaloblastic anemia should be polyneuropathy with macrocytosis, we would agree to that, widening our mind with other differential diagnosis of macrocytosis and polyneuropthy rather than one, megaloblastic anemia.

Writing this response was a teaching round for us, as you have lightened out so many points that would have also seemed unclear to others as well, and we thank you for giving us the opportunity for this response.

As you have mention Sir, writing a case report of a POEMS syndrome is indeed not everyone cup of tea, and at the same time solving and proving it is also not an easy task being a rare case with very few case reports worldwide. Hence reporting it is worthwhile, reminding us not to forget the other unusual differentials while making our diagnosis.

 
  References Top

1.Savage DG, Lindenbaum J, Stabler SP, Allen RH. Sensitivity of serum methylmalonic acid and total homocysteine determinations for diagnosing cobalamin and folate deficiencies. Am J Med 1994;96:239-46.  Back to cited text no. 1
    
2.Miest RY, Comfere NI, Dispenzieri A, Lohse CM, el-Azhary RA. Cutaneous manifestations in patients with POEMS syndrome. Int J Dermatol 2013;52:1349-56.  Back to cited text no. 2
    
3.Sasy RG. Waldenstrom macroglobulnemia presenting features and outcomes in a series with 27 cases. Br J Haematol 2001;115:575.  Back to cited text no. 3
    




 

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