Case Report
Open Access
Secondary Amyloidosis Involving the Bone Marrow:
A Rare Case Report
Anand Amrita1 and Agarwal Shilpi1
1Lady Hardinge Medical College, New Delhi, India
*Corresponding author: Anand Amrita, Lady Hardinge Medical College, New Delhi, India, Email:
@
Received: March 13, 2019; Accepted: March 26, 2019; Published: March 28, 2019
Citation: Anand A, Shilpi A (2019) Secondary Amyloidosis Involving the Bone Marrow: A Rare Case Report. J Emerg Crit Care Diagn Manag 2(1): 1-3.
Amyloidosis encompasses a heterogenous group of diseases
caused by extracellular deposition of fibrillary protein. Amyloid
deposits in AA Amyloidosis are composed mainly of serum amyloid
protein, an apolipoprotein of High Density Lipoprotein (HDL) that
serves as an acute phase reactant. Amyloid is usually not seen or
it is very sparsely presented in a Bone Marrow aspirate or biopsy.
Herein we discuss a case of 55 years old male with this rare
presentation of secondary amyloidosis, involving bone marrow.
Key Words: BM- Bone Marrow; CT Scan -Computed Tomography Scan; TLC- Total Leucocyte Count; HDL- High Density Lipoprotein
Key Words: BM- Bone Marrow; CT Scan -Computed Tomography Scan; TLC- Total Leucocyte Count; HDL- High Density Lipoprotein
A 54 year old male patient, known case of Chronic Liver
Disease being treated for Abdominal Koch’s for last 4 months
presented in the Medicine OPD with the complaint of persistent
abdominal distention for 2 months. It was associated with a
decreased oral intake, shortness of breath and a decreased
urine output. On general physical examination the patient had
icterus, gross ascites and hepatomegaly. There was no associated
splenomegaly or lymphadenopathy. The liver span was 13cm.
Contrast Enhanced CT Scan revealed hepatomegaly with acute
or chronic liver disease with findings suggestive of possibility
for Amyloidosis, leading to Portal Hypertension that cannot
be ruled out. X-Ray Pelvis did not reveal any lytic lesions. Liver
function Tests were deranged showing the following parameters:
total Bilirubin raised to 2mg/dl (n= 0.2-1.2mg/dl); Alkaline
Phosphatase raised to 953U/L (n= 44-147 U/L) and Gamma
Glutamyl Transferase raised to 1956 U/L (n= 44-147U/L). Renal
Function Tests were deranged showing the following parameters.
S. Urea 139mg/dl (n= 7-20mg/dl), S. Creatinine 4mg/dl (n=
0.6-1.2mg/dl), S. Uric acid 7mg/dl (n= 3.4-7mg/dl). Complete
Hemogram revealed Leucocytosis and Thrombocytopenia.
Hemoglobin was 11.6g/dl (n= 13.5-17.5g/dl), TLC 15.6 x 103/
microl (n= 4-11x103) and platelet count 97 x103/ microl (n= 1.5-
4x103). Ascitic Fluid Biochemistry revealed Adenine Deaminase
levels < 4 U/l with 2100 mononuclear cells/mm3 suggesting
availability of transudate.
Hence for confirmation a Bone Marrow Aspirate and Biopsy were done. Bone Marrow Aspirate smears showed cellular particles with normal maturation in Erythroid, Myeloid and Megakaryocyte series. There was no dysplasia seen amongst the hematopoietic cells. The myelogram was nRBC39 Myelocytes24 Metamyelocytes20 Stab07 Neutrophils02 Plasma cells 05 Lymphocytes03. However, an extensive deposition of a pink amorphous extracellular material between these hematopoietic cells was established (Figure1, Figure 2). In staining of the same material with Methyl Violet metachromatically positivity was assessed (Figure 3), which suggested Amyloid deposition.
Bone marrow trephine biopsy showed mildly hypocellular bone marrow with adequate representation of erythroid, myeloid and megakaryocyte series. In addition, there was presence of abundant extracellular eosinophilic amorphous material (probably amyloid) in the vessel wall and interstitium (Figure 4). This material was also metachromatically positive for methyl violet. Immunohistochemistry for Serum Amyloid, a Protein assay was done for confirmation, and the probe was reported as positive (Figure 5).
Hence for confirmation a Bone Marrow Aspirate and Biopsy were done. Bone Marrow Aspirate smears showed cellular particles with normal maturation in Erythroid, Myeloid and Megakaryocyte series. There was no dysplasia seen amongst the hematopoietic cells. The myelogram was nRBC39 Myelocytes24 Metamyelocytes20 Stab07 Neutrophils02 Plasma cells 05 Lymphocytes03. However, an extensive deposition of a pink amorphous extracellular material between these hematopoietic cells was established (Figure1, Figure 2). In staining of the same material with Methyl Violet metachromatically positivity was assessed (Figure 3), which suggested Amyloid deposition.
Bone marrow trephine biopsy showed mildly hypocellular bone marrow with adequate representation of erythroid, myeloid and megakaryocyte series. In addition, there was presence of abundant extracellular eosinophilic amorphous material (probably amyloid) in the vessel wall and interstitium (Figure 4). This material was also metachromatically positive for methyl violet. Immunohistochemistry for Serum Amyloid, a Protein assay was done for confirmation, and the probe was reported as positive (Figure 5).
Figure 1:Bone marrow aspirate 400x Wright- Giemsa
Figure 2:Bone marrow aspirate (1000x) Wright - Giemsa
Figure 3:Methyl violet bone marrow aspirate 400x
Figure 4:Bone marrow Biopsy 400x H&E
Figure 5:Serum Amyloid associated protein IHC-BMB 400x
Serum Protein Electrophoresis with immunofixation revealed
a polyclonal increase in the Kappa Light chain to 79.20 mg/dl and
Lambda Light chain to 93.60 mg/dl. The b2 microglobulin levels
were increased to 9687ng/ml. Myeloma protein panel showed an
albumin level of 1.73 g/dl. No Myeloma Band was detected, hence
ruling out Multiple Myeloma.
So, a clinicopathological diagnosis of Secondary Amyloidosis involving the bone marrow was made.
So, a clinicopathological diagnosis of Secondary Amyloidosis involving the bone marrow was made.
Amyloidosis encompasses a heterogeneous group of diseases,
caused by the extracellular deposition of autologous fibrillar
protein, which aggregates into a 3-D beta-lamina disposition that
impairs normal organ function [1].
Amyloid deposits in AA Amyloidosis are composed mainly of Serum Amyloid A Protein, an apolipoprotein of HDL that serves as an acute phase reactant [2].
Amyloid is usually not seen or it is very sparsely presented in aspirate or biopsy. Much of our information regarding amyloid involving bone marrow is based on primary amyloidosis.
On extensive research we could find a single study by Sungur et al, who reported secondary amyloidosis involving bone marrow due to renal failure caused by Familial Mediterranean Fever [3].
The current study conducted bone marrow biopsy on 14 out of 39 patients. Amyloid deposition was seen in 11/14 (78.6%) of the patients.
Secondary amyloidosis involving bone marrow amyloid deposition is very unusual and is rarely reported on the literature.
Amyloid deposits in AA Amyloidosis are composed mainly of Serum Amyloid A Protein, an apolipoprotein of HDL that serves as an acute phase reactant [2].
Amyloid is usually not seen or it is very sparsely presented in aspirate or biopsy. Much of our information regarding amyloid involving bone marrow is based on primary amyloidosis.
On extensive research we could find a single study by Sungur et al, who reported secondary amyloidosis involving bone marrow due to renal failure caused by Familial Mediterranean Fever [3].
The current study conducted bone marrow biopsy on 14 out of 39 patients. Amyloid deposition was seen in 11/14 (78.6%) of the patients.
Secondary amyloidosis involving bone marrow amyloid deposition is very unusual and is rarely reported on the literature.
ReferencesTop
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- Malle E, Sodin-Semrl S, Kovacevic A. Serum amyloid A: an acute-phase protein involved in tumour pathogenesis. Cell Mol Life Sci. 2009;66(1):9-26. doi: 10.1007/s00018-008-8321-x
- Sungur C, Sungur A, Ruacan S, Arik N, Yasavul U, Turgan C, et al. Diagnostic value of bone marrow biopsy in patients with renal disease secondary to familial Mediterranean fever. Kidney Int. 1993;44(4):834-836.