|Year : 2021 | Volume
| Issue : 1 | Page : 48-50
Osteoblasts to the Rescue – A Rare Case of Secondary Myelofibrosis
Lawanya Gunaseelan1, Eliz Thomas1, Prasanna N Kumar1, Sujaya Menon2
1 Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India
2 Department of Medicine, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India
|Date of Submission||28-Jul-2020|
|Date of Decision||11-Sep-2020|
|Date of Acceptance||08-Dec-2020|
|Date of Web Publication||15-Mar-2021|
Dr. Eliz Thomas
Department of Pathology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore - 641 004, Tamil Nadu
Source of Support: None, Conflict of Interest: None
A 36-year-old female patient presented with joint pain, generalized weakness, and bleeding gums. There was no significant history of other illnesses or intake of drugs. Examination revealed pallor and splenomegaly. Peripheral smear examination showed pancytopenia. A large number of osteoblasts and a few scattered osteoclasts were seen in the bone marrow aspirate. The trephine biopsy revealed marrow fibrosis. A suggestion to investigate for hyperparathyroidism was made. Serum calcium, ionized calcium, and PTH were increased. 99m Tc-Sestamibi scan was done and adenoma was detected in the right inferior parathyroid and subsequently excised. Postsurgery, hematological parameters and bone markers improved. Follow-up bone marrow biopsy revealed hypercellular marrow with marked regression of fibrosis. Splenomegaly was reversed. We concluded that pancytopenia was due to bone marrow fibrosis, resulting from primary hyperparathyroidism. It is important to consider secondary causes of myelofibrosis in the appropriate clinical setting.
Keywords: Osteoblasts in the marrow, secondary myelofibrosis, marrow fibrosis
|How to cite this article:|
Gunaseelan L, Thomas E, Kumar PN, Menon S. Osteoblasts to the Rescue – A Rare Case of Secondary Myelofibrosis. J Appl Hematol 2021;12:48-50
| Background|| |
Primary myelofibrosis is commonly a disease of the elderly. Secondary myelofibrosis occurs due to various pathological processes that involve the bone marrow such as malignancy, infection, metabolic disorders, radiation, and drugs. Hyperparathyroidism as a precipitating factor for myelofibrosis has rarely been reported.,It is important to consider secondary causes of myelofibrosis in the appropriate clinical setting. Our patient improved, similar to others after surgery. Diagnosis was confirmed when fibrosis in the bone marrow reversed after surgery.
| Case Report|| |
We report a 36-year-old female who presented with joint pain, generalized weakness, and bleeding gums. There was no significant history of other illnesses or intake of any drugs. Examination revealed pallor and massive splenomegaly. Peripheral smear examination showed pancytopenia. Bone marrow aspiration showed a large number of osteoblasts and a few scattered osteoclasts [Figure 1]. The trephine biopsy revealed marrow fibrosis of grade 3 [Figure 2]. On the basis of the presence of abundant osteoblasts and fibrosis, a suggestion to investigate for hyperparathyroidism was made. On further investigation, serum calcium, serum ionized calcium, and PTH were found to be increased [Table 1]. 99m Tc-Sestamibi scan was done, and adenoma was detected in the right inferior parathyroid and was subsequently excised [Figure 3].
As early as 6 months postsurgery, hematological parameters and bone markers improved without any intervention. During the follow-up, bone marrow biopsy revealed hypercellular marrow and marked regression of fibrosis. The spleen also gradually decreased in size. We concluded that the pancytopenia was because of bone marrow fibrosis resulting from primary hyperparathyroidism.
All the causes for pancytopenia were ruled out one by one. Due to the presence of osteoblasts and osteoclasts in the marrow aspirate, along with fibrosis in the biopsy, a suggestion to investigate for hyperparathyroidism was made.
Fluids, diuretics, bisphosphonates, and other supportive measures were administered to treat hypercalcemia. Thalidomide and prednisolone were given in tapering doses.
The parathyroid adenoma was excised and confirmed by histopathology.
Outcome and follow-up
Six months post-surgery, hematological parameters and bone markers improved [Table 2]. Bone marrow biopsy revealed hypercellular marrow and marked regression of fibrosis [Figure 4]. The spleen gradually decreased in size.
|Figure 4: Bone marrow biopsy post surgery shows presence of hematopoietic elements|
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| Discussion and Conclusion|| |
Pancytopenia is a common hematological entity, and the various causes have to be evaluated for. Myelofibrosis is one such cause and primary myelofibrosis is known to occur in the elderly. Secondary myelofibrosis can be due to various etiologies such as drugs, neoplasms, collage diseases, hyperparathyroidism, and renal osteodystrophy. Literature says that asymptomatic primary hyperparathyroidism is rare.,,, Our patient had features of advanced hyperparathyroidism such as elevated blood calcium levels, PTH levels, ALP levels and radiological evidence of subperiosteal bone resorption and osteopenia. After the curative parathyroid surgery, improvement in hematological and metabolic parameters is very suggestive of marrow fibrosis due to primary hyperparathyroidism.
Learning points/take home messages
- Evaluation for secondary myelofibrosis in correlation with clinical and biochemical parameters is of paramount significance
- Hyperparathyroidism must be considered with the presence of osteoblasts in the marrow aspirate.
Ethics approval and consent to participate
Ethics approval was obtained and consent to participate was taken from the patient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]