Serous Fat Atrophy of Marrow: A Case Report

 

Dr. Singh U.R.1, Dr. Sutrakar S.K.2, Dr. P.C. Kol1, Dr. Sunil Rao3, Dr. Ravishanker Shrivastava4

1Associate Professor, Department of Pathology, S.S. Medical College and Asso. S. G.M. Hospital Rewa (M.P.)

2Assistant Professor, Department of Pathology, S.S. Medical College and Asso. S.G.M. Hospital Rewa (M.P.)

3Assistant Professor, Department of Pediatrics, S.S. Medical College and Asso. S.G.M. Hospital Rewa (M.P.)

4Resident Medical Officer, Department of Pathology, S.S. Medical College and Asso. S.G.M. Hospital Rewa (M.P.)

 

 

ABSTRACT:

Serous fat atrophy or gelatenous transformation of marrow (GMT) is a rare disorder of unknown pathogenesis characterized by cell atrophy, focal loss of haematopoietic cells and deposition of extra cellular material. This also has been termed as "Starvation Marrow". It is reversible with resolution of nutritional problem. It is an epiphenomenon associated with extreme malnutrition and weight loss. Its etiology is age related. It may be found in Kwashiorkar, anorexia nervosa, AIDS, cachexia, carcinoma, lymphoma, marrow damage caused by toxin, radiation or chemotherapy, it is sometimes seen early in course of aplastic anemia when it’s probably indicate recent or ongoing marrow damage.

 

KEYWORDS: Atrophy, Extra cellular material, Bone marrow, Kwashiorkar, AIDS.

 

 

Case Report:

Serous fat atrophy or gelatenous transformation of marrow (GMT) is a rare disorder of unknown pathogenesis characterized by cell atrophy, focal loss of haematopoietic cells and deposition of extra cellular matrial1. This also has been termed as "Starvation Marrow"2.It is reversible with resolution of nutritional problem3.It is an epiphenomenon associated with extreme malnutrition and weight loss. Its etiology is age related. It may be found in Kwashiorkar, anorexia nervosa, AIDS, cachexia, carcinoma, lymphoma, marrow damage caused by toxin, radiation or chemotherapy,4 it is some times seen early in course of aplastic anemia when its probably indicate recent or on going marrow damage.

 

A 13 years old female patient presented in paediatric OPD with severe malnutrition pallor and hepatomegaly. Reviewing her history she belonged to very low socioeconomic status. On admission she appeared very thin, her body weight was 27 kg (<3rd percentile) and her body height was 155.2c.m (75th-90th percentile). She has no palpable lymph node. She had a heart rate of 50-60/min. per abdomen examination revealed mild hepatomegaly. Lab exam was done revealing Hb level of 7gm%, Hct of 22%, RBC count was 2.8 million/L, MCV-78pg, MCH-25pg, MCHC-32gldl, WBC count showed 2900/L with 40% neutrophils, 52% lymphocytes, 7% monocytes and 1% eosinophils, platelets count was 80,000/L, peripheral smear examined showed a pancytopenic picture with microcytic hypochromic anemia, BUN was 21 mg/dl, creatinine was 2.7 gm/dl, blood sugar was 95 mg/dl, total serum protein was 4.5 gm/dl, SGOT and SGPT were within normal range cholesterol level was 232 mg/dl.

 

 

To evaluate Pancytopenia bone marrow aspiration was carried out the marrow smear showed hypo cellular fragments of marrow with decreased fat cells and presence of extra cellular pink purple amorphous material. "Serous atrophy of marrow" was suggested with advice for trephine biopsy. The bone marrow biopsy showed hypo cellular marrow with decreased hematopoiesis, decrease in fat cell number as well as size and replacement of marrow space by amorphous eosinophilic extracellular ground substance. A diagnosis of "serous fat atrophy of marrow was rendered". (Ref. Fig 1 and 2 ).The above mentioned patient received nutritional and supportive treatment.

 

Follow up blood count after 5 weeks, showed Hb-9.5gm% and MCV 90.9fl, WBC count of 4200/L, platelet count  of 2,22,000/L, PS examination showed normocytic normochromic anemia. The patient condition improved with nutritional support.

 

Fig. 1 (H and E 40 x)

Section of marrow demonstrating hypoplasia, decreased fat spaces and replacement of bone marrow by amorphous ground substance.

Fig. 2 (H and E 400 x)

Shows section of marrow demonstrating deposition of amorphous eosinophilic material in marrow space with decreased number as well as size of fat cells

 

DISCUSSION:

Serous fat atrophy of marrow should be considered in evaluating patients with Pancytopenia, especially who are having severe malnutrition. The marrow is hypo cellular and hematopoiesis is decreased with reduction predominantly of erythroid component, plasma cells lymphocytes and phagocytes were relatively increased.5 The fat cells are decreased and are smaller than normal.6 There is deposition of gelatinous material which is amorphous faintly eosinophilic and extra cellular. This substance is mainly hyaluronic acid in patient with starvation and anorexia. Its resemblance to deposit of serum or plasma protein have led to the term "serous fat atrophy" but it is a descriptive term rather than expression of a known pathophysiologic mechanism.7  Almost all the patients in GMT are found to be anemic.

 

Most authors speculate that deposition of hyaluronic acid in GMT lesion may contribute to development of anemia as recent studies have also indicated that natural polysaccharide are not suitable for haematopoietic proliferation.1,2,8    GMT has been commonly reported in case of malnutrition secondary to starvation and anorexia nervosa. Association of GMT with chronic mal/under nutrition suggested that the gelatinous transformation of marrow is caused by excessive production of mucopolysaccharose of ground substance to compensate for mobilization of marrow fat which may have occurred to meet energy requirement.9   Reversibility of GM to normal marrow have been reported after clinical improvement from starvation state indicating GMT is not a permanent phenomenon. This may be due to limited cell necrosis (if at all) as marrow's cellularity also decreased due to lack of synthesis of growth factors secondary to starvation/infections.10

 

GMT have also been reported to be associated with malignancies lymphoma, carcinoma and multiple myeloma  have been found in association with GMT in one series.2 It has been suggested that malignant cells might produce or stimulate the production of hyaluronic acid leading to gelatinous transformation. It if felt that hyaluronic acid was a transient event terminated by filling up the marrow space by either proliferating haematopoietic or leukaemic cells and so evidently the deposition of hyaluronic acid was a result rather than cause.5

 

In patients with AIDS the gelatinous material contains large amount of sulfated glycosaminoglycon in addition to hyaluronic acid.11 GMT needs to be differentiated from nuclear threading of hemopoietic cells caused by smearing artifact/delay in preparing the smear by showing signet ring type fat cells in the former and under cognizable. Morphology of hemopoietic cells in letter marrow edema is found in hypo cellular areas with a low content of hematopoiesis cells. In contrast to GMT, marrow edema shows fat cells of normal size surrounded by Alcian blue negative substance amyloid which stains with Congo red.2 Based on the heterogeneity of associated clinical condition, GMT, out as an indicator of severe illness and not of particular disease. The wide range of associated disease with GMT only indicate it to be result of basic bio regulatory process that are activated in different pathologic condition which led to morphologically similar lesion in bone marrow, gelatinous transformation of marrow is a reversible process if underlying disorder can be eliminated.

 

CONCLUSION:

In conclusion we present a case of serous fat atrophy of marrow due to severe malnutrition. The patient improved following supportive and nutritional supplement. Thus a diagnosis of serous fat atrophy of marrow should be considered in evaluating patients with Pancytopenia especially who are having severe malnutrition.

 

Abbreviations:

    GMT - Gelatinous transformation of marrow

    AIDS – Acquired Immunodeficiency Syndrome

    BUN – Blood urea nitrogen

    MCV – Mean Corpuscular Volume

    MCH – Mean Corpuscular Hemoglobin

    MCHC – Mean Corpuscular Hemoglobin Concentration.

 

REFERENCE:

1.     Clarke BE, Brown DS, Xipell JM. Gelatinous transformation of bone marrow. Pathology  1983:15;85-82.

2.       Bohm J. Gelatinous transformation of bone marrow-the spectrum of underlying disease. AMJ Surg. Pathol 2000,24:56;65.

3.       Basu S, Mitra S, Matwaha RK, Garewal G. Gelatinous transformation of bone marrow. Indian J Pathol Microbiol 1997:40;385-4.

4.       Seaman JP, Kjeldrbeig CR, Linked A,. Gelatinous transformation of bone marrow. Hum Pathol 1978;9:685-92.

5.       R. Sen, S. Singh, A. Gupta, J. Sen. Clinical profile in gelatinous bone marrow transformation. JAPI Vol. 57, June 2003:p-586-587.

6.       Abella E, Fellu E, Granda Imilla F. Orall A, Ribera JM, Sarrchez Planell L, Berga Li, Revestor JC, Rozman C. Bone marrow changes in anorexia nervosa are corelated with the amount of weight loss and not with other clinical findings. Am Iclin Pathol 2002; 48:582-8.

7.       Diane C, Farhl. Bone marrow. Chapter 20;p-846-847.

8.       Tavassoll M, Estlund TD,YamL, Neuman RS, Finkd H. Gelatinous transformation of bone marrow in prolonged self induced starvation. Scand I Haematol 1976;16:311-9.

9.       Tavassoll M. Differential response of bone marrow of extramedullaryadipose cells to starvation. Experimedia 1974;30:424-25.

10.    Mant MJ, Faraghat BS. The haematology of anorexia nervosa. Bt. J. Macmatal 1972;23:737-49.

11.    Mahtak K, Uascon P, Robboy S. The gelatinous bone marrow in patients with AIDS. Evidence of excess sulfatul glycoaminoglycon. Arch Pathol Lab. Med. 1992;116:504-508. 

 

 

Received on 01.09.2012

Modified on 22.06.2013

Accepted on 10.07.2013

© A&V Publication all right reserved

Research J. Pharmacology and Pharmacodynamics. 5(5): September–October 2013, 302-304