LETTER TO THE EDITOR
Year : 2014 | Volume
: 19 | Issue : 2 | Page : 168--169
Writing poems or on POEMS is not everybody's cup of tea
Lalit S Raut
Leukemia/Bone Marrow Transplant Unit, Department of Medicine, Vancouver General Hospital, Vancouver, British Columbia, Canada
Lalit S Raut
58, Modern Housing Society,Pratap Nagar, Nagpur - 440022, Maharashtra
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Raut LS. Writing poems or on POEMS is not everybody's cup of tea.J Mahatma Gandhi Inst Med Sci 2014;19:168-169
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Raut LS. Writing poems or on POEMS is not everybody's cup of tea. J Mahatma Gandhi Inst Med Sci [serial online] 2014 [cited 2020 Aug 10 ];19:168-169
Available from: http://www.jmgims.co.in/text.asp?2014/19/2/168/138449
I would like to congratulate Tiewsoh et al. for the interesting case report.  Indeed, the clinical presentation of this patient with megaloblastic anemia and peripheral neuropathy could have mislead anybody. However, in my opinion, there are few points which need more clarification.
It would be interesting to know the details of the initial presentation and the investigations done at that time. Hemoglobin of 10 g% with mean corpuscular volume (MCV) of 100 fl with neuropathy does not confirm the diagnosis of megaloblastic anemia. MCV of >120 is a more common finding in megaloblastic anemia than MCV of 100.  Hypersegmented neutrophils are very subjective finding occurring with a number of causes. Further investigations would have helped to establish the diagnosis. Moreover, megaloblastic anemia is a syndromic diagnosis requiring further evaluation for the cause, especially in this 57-year-old otherwise healthy man. I assume that his initial presentation would have been to other physician or hospital and hence the full workup is not mentioned in this case report.What kind of skin hyperpigmentation did the patient present with? It would have been interesting to see the clinical photograph of the skin hyperpigmentation which would have been more informative to the readers. Skin hyperpigmentation is also seen in megaloblastic anemia especially the pigmentation of skin creases and nail beds. The skin may reveal a diffuse, brownish pigmentation, or abnormal blotchy tanning. The author mentions that the hemoglobin at the time of second presentation was 13g/dl with normocytic normochromic peripheral blood picture. The improvement in the hemoglobin and change from macrocytic to normocytic blood film probably suggests that he had some degree of vitamin B12 and/or folate deficiency which got corrected with vitamin supplementation. It is quite likely that initially, he had some degree of megaloblastic anemia and in addition he developed POEMS. The recovery from neuropathy of megaloblastic anemia can be extremely slow if the treatment is delayed. In this case, the patient was symptomatic for 9 months before being diagnosed and treated for megaloblastic anemia.  Thus, the background existence of megaloblastic anemia cannot be ruled out.The author states "the serum protein electrophoresis done was suggestive of prominent M-spike (7.7%, 0.60 g/dl) with a positive lambda light chain." In the discussion section, the author further mentions "serum electrophoresis is needed to detect the classical λ-restricted M protein." I think these both statements are misleading. It's surprising to get a report of positive lambda light chain based on serum protein electrophoresis which cannot tell us about the nature of the monoclonal protein. Hence, we do immunofixation and there is no mention of this investigation anywhere raising doubt on finding the lambda chain restricted monoclonal protein. In the light chain only disease, a clonal band is observed only in urine with no clear peak in serum. Hence, immunofixation should also be carried on the urine sample. Serum free light chain assay would have been useful in this setting. The λ chain clonality could have also been established by immunostaining using κ/λ staining or by flow cytometry in the bone marrow (BM) sample.  The case report fails to explain how the λ-restricted M protein was detected.In the discussion the author writes "multiple myeloma seemed unlikely since no oligolonal band were seen on serum electrophoresis." This is confusing to the readers as the serum protein electrophoresis clearly shows the M band. In my opinion, symptomatic multiple myeloma is ruled by the fact that the M protein in serum is <30 g/L, marrow showed <10% plasma cells and there was no evidence of CRAB (acronym for hypercalcemia, renal dysfunction, anemia and bone osteolytic lesions). The author states that the presence of polyneuropathy made multiple myeloma less likely. The fact is multiple myeloma can present with polyneuropathy in almost 30% cases.  The author's explanation for excluding multiple myeloma is incomplete and inappropriate.In the discussion section, the author adds "Waldenstorm's macroglobulinemia (WM) shows M protein on electrophoresis, they are not associated with end organ damage or organomegaly." According to the international working group on WM, WM is defined as a distinct clinicopathologic entity resulting from the accumulation, predominantly in the BM, of clonally related lymphocytes, lymphoplasmacytic cells, and plasma cells that secrete a monoclonal immunoglobulin M (IgM) protein. WM presents with anemia, symptoms of hyperviscosity and can cause organ dysfunction.  The letter M in "M protein" stands for monoclonal protein and not IgM. In WM the M protein is IgM. The author's statement is not fully informative.Thrombocytosis is defined as platelets of 450,000/mm 3 . Patient's platelet count was 502,000/mm 3 and is one of the minor criteria for the diagnosis of POEMS. The authors neglected this point and mentioned only three minor criteria. In fact patient fulfilled four minor diagnostic criteria for POEMS.The author concludes that all cases of polyneuropathy and megaloblastic anemia should be looked into for plasma cell disorders. I would like to refine this statement by saying that all patients presenting with macrocytosis and peripheral neuropathy should be investigated for a number of hematological causes such as megaloblastic anemia, multiple myeloma, amyloidosis, and POEMS. Even myelodysplastic syndrome can present with peripheral neuropathy as an autoimmune phenomenon.
Sir, in the end I would like to praise the authors for their efforts. I feel this case report certainly would have been written in a better way and the manuscript should have been reviewed properly by hematologist. Though interesting this case report exposes the lack of background knowledge of the subject and lacks the much needed final touch.
|1||Tiewsoh I, Singh V, Jajoo UN. Megaloblastic anemia with peripheral neuropathy, a misleading initial presentation in POEMS syndrome: A case report. J Mahatma Gandhi Inst Med Sci 2014;19:55-8.|
|2||Savage DG, Ogundipe A, Allen RH, Stabler SP, Lindenbaum J. Etiology and diagnostic evaluation of macrocytosis. Am J Med Sci 2000;319:343-52.|
|3||Antony AC. Megaloblastic anemias. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, editors. Hematology: Basic Principles and Practice. 6 th ed. Philadelphia: Churchill Livingstone Elsevier; 2012. p. 473-503.|
|4||International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: A report of the International Myeloma Working Group. Br J Haematol 2003;121:749-57.|
|5||Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML, et al. Clinicopathological definition of Waldenstrom's macroglobulinemia: Consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. Semin Oncol 2003;30:110-5.|