Bone Marrow Sarcoidosis With Immune Thrombocytopenic Purpura

Global Journal of Pathology & Laboratory Medicine
Volume 2, Issue 3, January 2024
Received: December 09, 2024, Reviewed: December 20th, 2024, Accepted: December 26th, 2024, Published: January 02, 2025

Unified Citation Journals, Pathology 2023, 2(3) 1-7; https://doi.org/10.52402/Pathology222
ISSN 2754-0952Global Journal of Pathology & Laboratory Medicine
Unified Citation Journals, Pathology 2024, ISSN2754-0952

Presented in 14th Emirates Pathology, Digital Pathology & Cancer Conference Holiday Inn Dubai, UAE & Virtual
Authors Names: Yousef Assy M.D. • Yamama H. Mahamid •Roaa Badran M.D. •Waheeb Alturi M.D. • Hammodeh Abdel Hadi

Research interest: History & Philosophy of Science Inst.

Biography:
Yousef Assy, M.D. A graduate of Iuliu Hațieganu University of Medicine and Pharmacy, in Cluj Napoca, Romania.
Research assistant in Tel Aviv University, at the Faculty of Humanities, History & Philosophy of Science Inst.

Yamama H. Mahamed: senior student at the faculty of medicine, at Al Quds University of Jerusalem.

Roaa Badran M.D. a graduate of the faculty of medicine, at Al Quds University, Jerusalem.

Waheeb Alturi M.D. a graduate from the faculty of medicine, at Nicolae Testemitanu
State University of Medicine and Pharmacy, Moldova.

Hammodeh Abdel Hadi: a graduate from the faculty of medicine, at Nicolae Testemitanu
State University of Medicine and Pharmacy, Moldova.

 

Abstract
Sarcoidosis is a complex multi-system disorder mainly characterized by the formation of noncaseating granuloma at various sites of the body. Bone marrow sarcoidosis with immune thrombocytopenic purpura presents an exceedingly rare case that manifests with hematological, lymphatic, and musculoskeletal symptoms. A previously healthy 36-year-old man presented with flu-like symptoms, widespread ecchymosis, unexplained bruising, easy gum bleeding, and general fatigue. His complete blood count revealed a low platelet count. This report presents a case of sarcoidosis associated with immune thrombocytopenic purpura, refractory to multiple first-line
therapies, including steroids, but successfully managed with a thrombopoietin receptor agonist.

Introduction
Sarcoidosis is a condition that causes the growth of noncaseating granulomas in multiple organs, with an unknown cause. It can affect the lungs, muscles, skin, eyes, lymph nodes, blood, and kidneys. It is seen in people of all ages and races, but it is more common among African Americans and Scandinavians aged 30- 50 years [1]. In the early stages, it may not cause any symptoms, but when symptoms appear, they can include a persistent dry cough, eye or skin problems, swollen lymph nodes, fatigue, weight loss, fever or night sweats, and erythema nodosum [2]. Hematologic abnormalities, such as anemia and lymphopenia, are common, but immune thrombocytopenia is rare [2]. Renal manifestations are also rare, but when they occur, they usually affect the tubules rather than the glomeruli.

The diagnosis of sarcoidosis is based on three criteria: consistent clinical and radiological findings, histological confirmation of non necrotizing inflammation that is granulomatous in various tissues, and the exclusion of other causes of granulomatous disease. Some clinical manifestations, such as Löfgren’s syndrome, lupus pernio, and Heerfordt’s syndrome, are specific to sarcoidosis and do not require histological confirmation. Although the patient’s bone marrow was affected by sarcoidosis, their chest roentgenograms may have remained unremarkable; hence, the diagnosis was supported by the usual histology of noncaseating granulomas in the bone marrow and the rigorous exclusion of infectious illness. Diagnosis can be challenging, but high levels of angiotensin-converting enzyme are often present at the time of diagnosis [3]. Treatment options for sarcoidosis aim to lower the risk of morbidity and mortality and improve quality of life. Treatment decisions should consider organ involvement, the risk of severe morbidity, and the impact of the disease on quality of life [4].
Glucocorticoids are the first-line treatment for symptomatic illness and are effective [4].

Case Presentation
A 36-year-old male patient with a free medical history presented to the hospital complaining of
fever, flu-like symptoms, and generalized weakness, in addition to associated ecchymosis and easy
bruising of his body after minimal trauma. He denied any history of weight changes, chronic cough,
palpable lymphadenopathy, skin rash, or bleeding dyscrasias. A complete blood count showed a
platelet count of 8 K/μL, and blood film consistent with immune thrombocytopenic purpura (ITP). A
bone marrow biopsy revealed a slightly hypocellular marrow with adequate megakaryocytes,
consistent with the peripheral destruction of platelets, without any granulomas. No malignant cells
were identified. One month later, an X-ray was done showing bilateral mediastinal lymphadenopathy, as seen in the

figure below:

Figure 1: An image of the chest X-ray demonstrating bilateral mediastinal lymphadenopathy.

Table 1: Laboratory findings
The patient was started on Eltrombopag 25 mg. Later, the dose was increased to 50 mg for more
efficacy, the PLT count improved to 130 K/μL. In this case, sarcoidosis was diagnosed approximately
one month after the initial diagnosis of ITP.

Discussion
This case report presents a rare convergence of ITP and bone marrow sarcoidosis. Comprehensive
history-taking and physical examination remain vital for sarcoidosis diagnosis. Our patient presented
with fever, flu-like symptoms, ecchymosis, and bruises after minor trauma without any other complaints. A bone marrow biopsy showed a slightly hypocellular marrow with adequate megakaryocytes, consistent with peripheral destruction of platelets and lymph nodes. CD3, CD20, and Terminal deoxynucleotidyl transferase expression levels were evaluated and showed no evidence of malignancy. Bone marrow biopsies, along with a CT scan, revealed sarcoidosis. The most common hematologic abnormality associated with sarcoidosis is anemia, including iron deficiency, hemolysis, and anemia of chronic disease. The association with thrombocytopenia is rare [5], but it
has been documented. In a survey of 381 cases of thrombocytopenic purpura, five patients (1.0%)
– defined as a had sarcoidosis, while 2.0% of 324 patients with sarcoidosis had thrombocytopenia ֲ
platelet count of less than 100,000 × 109/L-[6].

ITP correlated with sarcoidosis is thought to be caused by three primary mechanisms:
hypersplenism, bone marrow infiltration, and antibody-mediated platelet destruction [7]. Both
hypersplenism and bone marrow infiltration were present in our patient, which may have
contributed to his thrombocytopenia. This combination also occurred in another case, along with
focal segmental glomerulosclerosis [7].

The first-line therapy for thrombocytopenia and concomitant sarcoidosis is immune suppression
with steroids, as steroids block established autoimmunity and reduce the formation of granulomas in the bone marrow, which may increase the platelet count. A large study documented
20 individuals with sarcoidosis and immune thrombocytopenia, most of the patients had platelet
counts below 30 ×10^9/L and experienced overwhelmingly positive therapeutic outcomes after
therapy with prednisone at 1 mg/kg per day for at least three consecutive weeks with no severe
bleeding or fatalities [8].

Additionally, immune suppression is frequently used to treat
thrombocytopenia and sarcoidosis due to their links to autoimmune or inflammatory states. This
provides a treatment plan for a patient with ITP secondary to sarcoidosis with dual utility [9].

The treatment journey of the presented case showed a complex interplay of medical interventions aimed at addressing both ITP and sarcoidosis. The patient initially underwent conventional therapies (prednisolone 1mg/kg, IV dexamethasone 1000mg, IVIG 2g for five days, followed by IV rituximab 1g weekly for four weeks), yielding only partial improvements and transient effects. Following the diagnosis of sarcoidosis, treatment strategies shifted to include immunosuppressive agents such as cyclosporine 100mg P.O. for four months and mycophenolic acid 500mg P.O. BID for six months, which also failed to yield significant progress. Notably, eltrombopag (25mg-50mg) was the instrumental agent that improved the patient’s platelet counts. This case highlights the challenge of managing dual diagnoses and the importance of tailored therapeutic approaches. The evolving treatment path underscores the need for flexibility, considering the rarity of concurrent conditions and the potential for unconventional solutions to achieve positive patient outcomes. We recommend conducting more randomized controlled trials of the different strategies of bone marrow sarcoidosis treatment to minimize the uncertainty of the therapeutic agent’s positive
outcome.

Conclusions
Bone marrow sarcoidosis with ITP can pose both diagnostic and therapeutic challenges due to its low
occurrence. Our case describes management with several medical interventions, only to find the
best results with eltrombopag. A high index of suspicion should be maintained when dealing with patients with ITP who show diverse symptoms of multiple systems. Searching for lymphadenopathy and rash and conducting
ancillary tests, such as a bone marrow biopsy or CT scan, should help confirm or deny the diagnosis.

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   To citation of this article: Yousef Assy M.D. • Yamama H. Mahamid •Roaa Badran M.D. •Waheeb Alturi M.D. • Hammodeh Abdel Hadi, Global Journal of Pathology & Laboratory Medicine